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Rituximab: Rituxan®, Truxima®, Ruxience®, Riabni™

Policy Number: PH-90109

Intravenous

Last Review Date: 12/07/2023

Date of Origin: 7/20/2010

Dates Reviewed: 09/2010, 12/2010, 02/2011, 03/2011, 05/2011, 06/2011, 09/2011, 12/2011, 03/2012, 06/2012, 09/2012, 12/2012, 03/2013, 06/2013, 09/2013, 12/2013, 03/2014, 06/2014, 09/2014, 12/2014, 03/2015, 05/2015, 08/2015, 11/2015, 02/2016, 05/2016, 08/2016, 10/2016, 02/2017, 05/2017, 08/2017, 10/2017, 02/2018, 05/2018, 07/2018, 09/2018, 12/2018, 03/2019, 06/2019, 09/2019, 10/2019, 12/2019, 03/2020, 06/2020, 09/2020, 12/2020, 01/2021, 03/2021, 06/2021, 09/2021, 12/2021, 01/2022, 03/2022, 06/2022, 09/2022, 12/2022, 03/2023, 06/2023, 09/2023, 12/2023

FOR PEEHIP Members Only -Coverage excludes the provider-administered medication(s) outlined in this drug policy from being accessed through a specialty pharmacy. It must be obtained through buy and bill.

  1. Length of Authorization 1-5,23-25,34,44,62,80,94-98,102-104,108,115-118,128-130,133-138, 153,155

Coverage will be provided for 6 months (12 months initially for pemphigus vulgaris) and may be renewed, unless otherwise specified.

  • Maintenance therapy for oncology indications may be renewed for up to a maximum of 2 years, unless otherwise specified:
    • Adult Acute Lymphoblastic Leukemia (ALL) may be renewed for a maximum of 18 doses.
    • Mantle Cell Lymphoma may be renewed until disease progression or intolerable toxicity.
    • Hairy Cell Leukemia may be renewed for up to a maximum of 12 doses.
    • Induction/Consolidation of Pediatric B-Cell Acute Leukemia and Aggressive Mature B-Cell Lymphomas may NOT be renewed.
    • Pediatric Hodgkin Lymphoma may NOT be renewed.
  • Management of Immunotherapy-Related Toxicities:
    • Myositis/Myasthenia Gravis/Encephalitis may NOT be renewed.
    • Bullous Dermatitis may be renewed for a maximum of 18 months (4 total doses).
  • Relapse therapy for Pemphigus Vulgaris must be at least 16 weeks past a prior infusion.
  • Chronic Graft-Versus-Host Disease (cGVHD) may NOT be renewed.
  • Hematopoietic Cell Transplantation may NOT be renewed.
  • Lupus Nephritis may be renewed ONLY in patients experiencing a disease relapse.
  • Complications of Transplanted Solid Organ may NOT be renewed.
  1. Dosing Limits

A. Quantity Limit (max daily dose) [NDC Unit]:

  • Rituxan 100 mg/10 mL single-dose vial for injection: 12 vials per 28 day supply
  • Rituxan 500 mg/50 mL single-dose vial for injection: 8 vials per 28 day supply
  • Truxima 100 mg/10 mL single-dose vial for injection: 12 vials per 28 day supply
  • Truxima 500 mg/50 mL single-dose vial for injection: 8 vials per 28 day supply
  • Ruxience 100 mg/10 mL single-dose vial for injection: 12 vials per 28 day supply
  • Ruxience 500 mg/50 mL single-dose vial for injection: 8 vials per 28 day supply
  • Riabni 100 mg/10 mL single-dose vial for injection: 12 vials per 28 day supply
  • Riabni 500 mg/50 mL single-dose vial for injection: 8 vials per 28 day supply

B. Max Units (per dose and over time) [HCPCS Unit]:

Oncology Indications

Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Leukemia (SLL):

  • Initial therapy:
    • Loading dose: 100 billable units x 1 dose
    • Subsequent doses: 130 billable units every 28 days x 5 doses per 6 months
  • Renewal therapy: 130 billable units every 8 weeks

ALL

  • 100 billable units twice weekly x 18 doses

Hairy Cell Leukemia

  • 100 billable units weekly x 8 doses, 100 billable units every 14 days x 8 doses, then 100 billable units every 28 days x 4 doses

Histiocytic Neoplasms – Rosai-Dorfman Disease

  • 130 billable units weekly x 6 doses in a 6 month period

Pediatric Hodgkin Lymphoma

  • 100 billable units x 3 doses

Chronic Graft-Versus-Host Disease (cGVHD)

  • 100 billable units weekly x 8 doses

Hematopoietic Cell Transplantation

  • Initial dose: 100 billable units x 1 dose before transplant
  • Subsequent doses: 250 billable units x 3 doses after transplant

All other oncology indications:

  • Initial therapy: 100 billable units weekly x 8 doses per 6 months
  • Renewal therapy: 100 billable units x 4 doses per 6 months

Non-Oncology Indications

Rheumatoid Arthritis (RA):

  • 100 billable units every 14 days x 2 doses in an 18-week period

Multiple Sclerosis (MS):

  • 100 billable units every 14 days x 2 doses every 6 months

Pemphigus Vulgaris (PV):

  • Initiation: 100 billable units weekly x 4 doses in a 12 month period
  • Maintenance and Relapse: 50 billable units every 16 weeks

GPA(WG)/MPA:

  • Induction: 100 billable units weekly x 4 doses in a 20-week period
  • Initial Maintenance: 50 billable units x 2 doses in a 6 month period
  • Subsequent Maintenance: 50 billable units every 6 months

All other non-oncology indications:

  • 100 billable units weekly x 4 doses in a 6 month period

  1. Initial Approval Criteria 1-4

Coverage is provided in the following conditions:

For PEEHIP Members Only

  • Ruxience (rituxumab-pvvr) and Truxima (rituximab-abbs) are the preferred products and all other rituximab products are non-preferred. Patients must have tried and had an inadequate response or intolerance to, or a contraindication to both the preferred products, attributable to the biosimilar formulation, prior to consideration of a non-preferred rituximab product; OR patient is continuing treatment with a non-preferred rituximab product for an oncology indication; AND

For Commercial Members Only

  • Ruxience (rituxumab-pvvr) and Truxima (rituximab-abbs) are the preferred products. Patient must have tried and had an inadequate response or intolerance to, or a contraindication to Ruxience and Truxima attributable to the biosimilar formulation, prior to consideration of a non-preferred rituximab product including Rituxan (rituximab) and Riabni (rituximab-arrx). Patients currently on non-preferred therapies may complete their current course of treatment for the duration of the current precertification period; upon precertification renewal or restarting therapy, transition to a preferred product is required; AND
  • Patient is at least 18 years of age (unless otherwise specified); AND

Universal Criteria 1-4

  • Patient does not have a severe, active infection; AND
  • Patient has been screened for the presence of hepatitis B (HBV) infection (i.e., HBsAg and anti-HBc) prior to initiating therapy and patients with evidence of current or prior HBV infection will be monitored for HBV reactivation during treatment; AND
  • Patient has not received a live vaccine within 28 days prior to starting treatment and live vaccines will not be administered concurrently while on treatment; AND

Oncology Indications 1-5

  • Patient is CD20 antigen expression positive (excluding use for cGVHD, Hematopoietic Cell Transplantation, and Management of Immunotherapy-Related Toxicity); AND

Pediatric Mature B-Cell Acute Leukemia (B-AL) † Ф 1

  • Patient is at least 6 months of age; AND
  • Used in combination with chemotherapy for previously untreated disease

Adult* Acute Lymphoblastic Leukemia (ALL) ‡ 5,93

  • Patient has Philadelphia chromosome-positive (Ph+) disease; AND
    • Used in combination with a tyrosine kinase inhibitor (TKI)-based regimen; AND
      • Patient is <65 years of age without significant comorbidities; OR 
    • Used in combination with MOpAD (methotrexate, vincristine, pegaspargase, dexamethasone) for TKI-refractory disease; OR
  • Patient has Philadelphia chromosome-negative (Ph-) disease; AND
    • Used as a component of a multiagent chemotherapy

*NCCN recommendations for Adult ALL may be applicable to adolescent and young adult (AYA) patients within the age range of 15-39 years.

Central Nervous System (CNS) Cancers ‡ 5

  • Patient has leptomeningeal metastases from lymphomas§; OR
  • Patient has primary CNS lymphoma; AND
    • Used for induction therapy; AND
      • Used as a single agent OR in combination with a methotrexate-containing regimen, temozolomide, or lenalidomide¥; OR
      • Patient has CSF positive or spinal MRI positive disease§; OR
    • Used for consolidation (monthly maintenance) therapy; AND
      • Used as continuation of induction regimen in patients with complete response or complete response unconfirmed (CRu) to induction therapy; AND
        • Used as a single agent§; OR
        • Used on combination with high-dose methotrexate¥; OR
    • Used for relapsed or refractory disease; AND
      • Used as a single agent OR in combination with systemic therapy in patients with prior whole brain radiation therapy§; AND
        • Patient has CSF positive or spinal MRI positive disease; OR
      • Used as a single agent OR in combination with temozolomide, lenalidomide, or high-dose methotrexate¥

§ For intrathecal administration ONLY; ¥ For intravenous administration ONLY

Adult Hodgkin Lymphoma ‡ 5

  • Patient has nodular lymphocyte-predominant disease

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) † ‡ Ф 1-5

  • Used in combination with fludarabine and cyclophosphamide (FC) ; OR
  • Patient has disease without del(17p)/TP53 mutation; AND
    • Used as first-line therapy in combination with bendamustine (excluding use in frail patients); OR
    • Used as subsequent therapy in combination with one of the following:
      • Bendamustine (patients <65 years of age without significant comorbidities; excluding use in frail patients)
      • Idelalisib
      • Lenalidomide
      • Venetoclax; OR
  • Patient has disease with del(17p)/TP53 mutation; AND
    • Used as first-line therapy in combination with high-dose methylprednisolone; OR
    • Used as subsequent therapy in combination with one of the following:
      • Alemtuzumab
      • High-dose methylprednisolone
      • Idelalisib
      • Lenalidomide
      • Venetoclax; OR
  • Used as first-line therapy for histologic (Richter’s) transformation to diffuse large B-cell lymphoma; AND
    • Used in combination with cyclophosphamide, doxorubicin, and vincristine-based regimens or as a component of OFAR (oxaliplatin, fludarabine, cytarabine, and rituximab)

Waldenström Macroglobulinemia/Lymphoplasmacytic Lymphoma ‡ 5

Adult B-Cell Lymphomas † Ф 1-5,44  including, but not limited to, the following:

  • HIV-Related B-Cell Lymphomas
    • Disease is related to Burkitt lymphoma, diffuse large B-cell lymphoma (DLBCL), HHV8-positive DLBCL (not otherwise specified), or primary effusion lymphoma (PEL)
  • Burkitt Lymphoma
    • Used in combination with chemotherapy
  • Castleman Disease
    • Patient has multicentric disease; OR
    • Patient has unicentric disease; AND
      • Used as second-line therapy for relapsed or refractory disease; OR
      • Used for unresectable disease or symptomatic disease after incomplete resection
  • Diffuse Large B-Cell LymphomaФ
  • Low-Grade (grade 1-2) or Follicular Lymphoma Ф
  • Extranodal Marginal Zone Lymphoma (EMZL) of the Stomach & Nongastric Sites (Noncutaneous)
  • Nodal & Splenic Marginal Zone Lymphoma
  • High-Grade B-Cell Lymphomas
  • Mantle Cell Lymphoma
  • Histologic Transformation of Indolent Lymphomas to Diffuse Large B-Cell Lymphoma
  • Post-Transplant Lymphoproliferative Disorders (PTLD) (B-Cell type)

Primary Cutaneous B-Cell Lymphomas ‡ 5

Pediatric Aggressive Mature B-Cell Lymphomas (Primary Mediastinal Large B-Cell Lymphoma, Diffuse Large B-Cell Lymphoma, Burkitt Lymphoma, & Burkitt-like Lymphoma) † ‡ Ф 1,5,50,121

  • Patient is at least 6 months of age*; AND
  • Used in combination with chemotherapy

*Pediatric Aggressive Mature B-Cell Lymphoma may be applicable to adolescent and young adult (AYA) patients older than 18 years of age and less than 39 years of age, who are treated in the pediatric oncology setting.

Hairy Cell Leukemia ‡ 5

  • Used as a single agent; AND
    • Used for less than complete response or relapsed disease in patients unable to receive purine analogs (i.e., cladribine or pentostatin); OR
  • Used in combination with cladribine; OR
  • Used in combination with pentostatin; AND
    • Used for less than complete response or relapsed disease; OR
  • Used in combination with vemurafenib; AND
    • Used as initial therapy in patients unable to tolerate purine analogs (i.e., cladribine or pentostatin) including frail patients and those with active infection; OR
    • Used for less than complete response or relapse within 2 years of complete response following initial treatment with cladribine or pentostatin; OR
    • Used for progression after therapy for relapsed or refractory disease (if not previously given); OR

 

  • Used in combination with venetoclax; AND
    • Used for progression after therapy for relapsed or refractory disease; AND
      • Patient had disease resistance to BRAF inhibitor therapy

Histiocytic Neoplasms – Rosai-Dorfman Disease 5

  • Used as a single agent for nodal, immune-cytopenia, or immunoglobulin G4 (IgG4) diseases; AND
    • Used for symptomatic unresectable unifocal disease; OR
    • Used for symptomatic multifocal disease; OR
    • Used for relapsed/refractory disease

Pediatric Hodgkin Lymphoma 5,128

  • Patient is ≤ 18 years of age*; AND
  • Patient has nodular lymphocyte-predominant disease; AND
  • Used in combination with CVbP (cyclophosphamide, vinblastine, prednisolone); AND
  • Used as primary treatment for stage IA or IIA disease (incomplete resection and non-bulky disease)

*Pediatric Hodgkin Lymphoma may be applicable to adolescent and young adult (AYA) patients up to the age of 39 years.

Chronic Graft-Versus-Host Disease (cGVHD) ‡ 5,22-25,45

  • Patient is post-allogeneic hematopoietic cell transplant (generally 3 or more months); AND
  • Used as additional therapy in combination with corticosteroids; AND
  • Patient has no response (e.g., steroid-refractory disease) to first-line therapy options

Hematopoietic Cell Transplantation (HCT) 5

  • Used as conditioning for allogeneic transplant as part of a non-myeloablative regimen in combination with cyclophosphamide and fludarabine

Management of Immunotherapy-Related Toxicities ‡ 5,62

  • Patient has been receiving therapy with an immune checkpoint inhibitor (e.g., cemiplimab, nivolumab, pembrolizumab, atezolizumab, avelumab, durvalumab, ipilimumab, dostarlimab, nivolumab/relatlimab-rmbw, tremelimumab etc.); AND
    • Patient has encephalitis related to immunotherapy; AND
      • Patient is autoimmune-encephalopathy-antibody positive; OR
      • Patient has had limited to no improvement after 7 to 14 days on pulse-dose corticosteroids with or without intravenous immunoglobulin (IVIG); OR
    • Patient has bullous dermatitis related to immunotherapy; AND
      • Used as additional therapy for moderate (G2), severe (G3) or life-threatening (G4) disease; OR
    • Patient has moderate, severe, or life-threatening steroid-refractory myositis (proximal muscle weakness, neck flexor weakness, with or without myalgias) related to immunotherapy; AND
      • Used for significant dysphagia, life-threatening situations, or cases refractory to corticosteroids; OR
    • Patient has myasthenia gravis related to immunotherapy; AND
      • Used as additional therapy for severe (G3-4) disease that is refractory to plasmapheresis or IVIG

Non-Oncology Indications

  • Patient is not on concurrent treatment with another CD20-directed therapy, TNF-inhibitor, IL-inhibitor, biologic response modifier or other non-biologic agent (e.g., apremilast, abrocitinib, tofacitinib, baricitinib, upadacitinib, deucravacitnib, etc.); AND

Rheumatoid Arthritis (RA) † 1-4,46-49,112,113

  • Physician has assessed baseline disease severity utilizing an objective measure/tool; AND
  • Documented moderate to severe active disease; AND
  • Used in combination with methotrexate unless the patient has a contraindication or intolerance; AND
  • Patient tried and failed at least a 3-month trial with ONE oral disease modifying anti-rheumatic drug (DMARD) (e.g., methotrexate, azathioprine, auranofin, hydroxychloroquine, penicillamine, sulfasalazine, leflunomide, etc.); AND
  • Previous failure with one or more preferred TNF antagonists at least one of which should be a self-injectable; AND
  • Patient has not had treatment with rituximab in the previous 4 months

Pemphigus Vulgaris † Ф 1,10,11,35,36,38,61,80,114,139

  • Patient has a diagnosis of pemphigus vulgaris as determined by the following:
    • Patient has one or more of the following clinical features:
      • Appearance of lesions, erosions and/or blisters
      • Nikolsky sign (induction of blistering via mechanical pressure at the edge of a blister or on normal skin)
      • Characteristic scarring and lesion distribution; AND
    • Histopathologic confirmation by skin/mucous membrane biopsy; AND
    • Positive direct immunofluorescence (DIF) microscopy result OR the presence of autoantibodies as detected by indirect immunofluorescence (IIF) or enzyme-linked immunosorbent assay (ELISA); AND
  • Patient has moderate to severe disease as assessed utilizing an objective measure/tool (e.g.., PDAI, PSS, ABSIS, etc.); AND
  • Used in combination with glucocorticoids (e.g., prednisone, prednisolone, etc.); AND
  • Other causes of blistering or erosive skin and mucous membrane diseases have been ruled out

Granulomatosis with Polyangiitis (GPA) (Wegener’s Granulomatosis) and Microscopic Polyangiitis (MPA) † Ф 1-4,125

  • Patient is at least 2 years of age; AND
  • Used in combination with glucocorticoids (e.g., prednisone, methylprednisolone, etc.)

Thrombocytopenic Purpura ‡ 6-9,63,127

  • Diagnosis includes one of the following:
  • Primary thrombocytopenia or idiopathic (immune) thrombocytopenia purpura (ITP)
  • Evans syndrome; AND
  • Patient has previously failed or has a contraindication or intolerance to therapy with corticosteroids; AND
  • The patient has a platelet count ≤30 x 109/L OR 30 x 109/L to < 50 x 109/L with symptomatic bleeding or with increased risk for bleeding

Thrombotic Thrombocytopenic Purpura (TTP) ‡ 16-18,20,21,126

  • The patient has a platelet count ≤30 x 109/L OR 30 x 109/L to < 50 x 109/L with symptomatic bleeding or with increased risk for bleeding; AND
  • Patient has immune-mediated or acquired disease with ADAMTS13-deficiency; AND
  • Used in combination with corticosteroids and therapeutic plasma exchange (TPE); OR
  • Used as a single agent as prophylactic therapy for patients in remission

Multiple Sclerosis (MS) ‡ 144,148

  • Patient must have a confirmed diagnosis of multiple sclerosis (MS) as documented by laboratory report (i.e., MRI); AND
  • Patient has a diagnosis of a relapsing form of MS [i.e., relapsing-remitting MS (RRMS)*, active secondary progressive disease (SPMS)**, or clinically isolated syndrome (CIS)***]

*Definitive diagnosis of MS with a relapsing-remitting course is based upon BOTH dissemination in time and space. Unless contraindicated, MRI should be obtained (even if criteria are met). 148

Dissemination in time

(Development/appearance of new CNS lesions over time)

Dissemination in space

(Development of lesions in distinct anatomical locations within the CNS; multifocal)

  • ≥ 2 clinical attacks; OR
  • 1 clinical attack AND one of the following:
    • MRI indicating simultaneous presence of gadolinium-enhancing and non-enhancing lesions at any time or by a new T2-hyperintense or gadolinium-enhancing lesion on follow-up MRI compared to baseline scan
    • CSF-specific oligoclonal bands
  • ≥ 2 lesions; OR
  • 1 lesion AND one of the following:
    • Clear-cut historical evidence of a previous attack involving a lesion in a distinct anatomical location
    • MRI indicating ≥ 1 T2-hyperintense lesions characteristic of MS in ≥ 2 of 4 areas of the CNS (periventricular, cortical or juxtacortical, infratentorial, or spinal cord)

                           

**Active secondary progressive MS (SPMS) is defined as the following: 145,148-150

  • Expanded Disability Status Scale (EDSS) score ≥ 3.0; AND
  • Disease is progressive ≥ 3 months following an initial relapsing-remitting course (i.e., EDSS score increase by 1.0 in patients with EDSS ≤5.5 or increase by 0.5 in patients with EDSS ≥6); AND
  • ≥ 1 relapse within the previous 2 years; OR
  • Patient has gadolinium-enhancing activity OR new or unequivocally enlarging T2 contrast-enhancing lesions as evidenced by MRI

***Definitive diagnosis of CIS is based upon ALL of the following: 148

  • A monophasic clinical episode with patient-reported symptoms and objective findings reflecting a focal or multifocal inflammatory demyelinating event in the CNS
  • Neurologic symptom duration of at least 24 hours, with or without recovery
  • Absence of fever or infection
  • Patient is not known to have multiple sclerosis

Autoimmune Hemolytic Anemia (AIHA) 26-32

  • Patient has warm-reactive disease refractory to or dependent on glucocorticoids; OR
  • Patient has cold agglutinin disease with symptomatic anemia, transfusion-dependence, and/or disabling circulatory symptoms

Lupus Nephritis 115-117,132,153,155,159

  • Patient has disease that is non-responsive or refractory to standard first-line therapy (i.e., mycophenolate mofetil, mycophenolic acid, cyclophosphamide, or calcineurin inhibitors [e.g., tacrolimus]); AND
  • Used as a single agent OR as add-on therapy in combination with mycophenolate mofetil, mycophenolic acid, or cyclophosphamide

Myasthenia Gravis (unrelated to immunotherapy-related toxicity) 118-120,156

  • Patient has muscle-specific tyrosine kinase (MuSK)-antibody positive disease; AND
  • Patient is refractory to standard first-line therapy (e.g., glucocorticoids, azathioprine, mycophenolate mofetil, etc.)

Complications of Transplanted Solid Organ (kidney, liver, lung, heart, pancreas) in Adult and Pediatric* Patients 133-138

  • Used for suppression of panel reactive anti-human leukocyte antigen (HLA) antibodies prior to transplantation; OR
  • Used for treatment of antibody-mediated rejection of solid organ transplantation

*Note: There is no minimum age requirement for this indication

Neuromyelitis Optica Spectrum Disorder (NMOSD) 90-92,157,165

  • Patient has a confirmed diagnosis based on the following:
    • Patient was found to be seropositive for aquaporin-4 (AQP4) IgG antibodies; AND
      • Patient has at least one core clinical characteristic § (*Note: some core clinical characteristics require both clinical and typical MRI findings); AND
      • Alternative diagnoses have been excluded [e.g., myelin oligodendrocyte glycoprotein (MOG) antibody disease (MOGAD), multiple sclerosis, sarcoidosis, cancer, chronic infection, etc.]; OR
    • Patient is seronegative for AQP4-IgG antibodies OR has unknown AQP4-IgG status; AND
      • Patient has at least two core clinical characteristics § occurring as a result of one or more clinical attacks; AND
      • Patient has experienced ALL of the following:
        • At least 1 core clinical characteristic must be acute optic neuritis, acute myelitis, or area postrema syndrome
        • Fulfillment of typical MRI findings requirements for each area affected ψ; AND
      • Alternative diagnoses have been excluded [e.g., myelin oligodendrocyte glycoprotein (MOG) antibody disease (MOGAD), multiple sclerosis, sarcoidosis, cancer, chronic infection, etc.]; AND
  • Used as a single agent or in combination with immunosuppressive therapy (e.g., azathioprine, methotrexate, mycophenolate, etc.)

§ Core Clinical Characteristics of NMOSD 90,157

  • Acute optic neuritis
  • Acute myelitis
  • Acute area postrema syndrome (APS): episode of otherwise unexplained hiccups and/or nausea and vomiting (lasting for at least 48 hours or with MRI evidence of a dorsal brainstem lesion)
  • Acute brainstem syndrome other than APS
  • Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic lesion on MRI ¥
  • Acute cerebral syndrome with NMOSD-typical brain lesion on MRI §

ψ Typical MRI findings in NMOSD related to clinical presentation (T2 unless noted otherwise) 157

  • Optic neuritis: Normal cerebral MRI (or only nonspecific white matter lesions) OR longitudinally extensive optic nerve lesion (≥half of the length of the optic nerve or involving optic chiasm; T2 or T1/Gd)
  • Myelitis: Intramedullary lesion3 contiguous VS (LETM) OR focal atrophy3 contiguous VS in patients with a history of acute myelitis
  • Area postrema syndrome (APS): Lesion in the dorsal medulla oblongata/area postrema
  • Other brainstem syndrome: Periependymal brainstem lesion (4th ventricle)
  • ¥ Diencephalic syndrome: Periependymal lesion (3rd ventricle) OR hypothalamic/thalamic lesion
  • § Cerebral syndrome: Extensive periependymal lesion (lateral ventricle; often with Gd) OR long (>12 length), diffuse, heterogeneous or edematous corpus callosum lesion OR long corticospinal tract lesion (unilateral or bilateral, contiguously involving internal capsule and cerebral peduncle) OR large, confluent (unilateral or bilateral) subcortical or deep white matter lesion

*LETM = longitudinally extensive transverse myelitis lesions

Systemic Lupus Erythematosus

  • The patient’s disease is active; AND
  • The patient has a history of positive autoantibody test results [positive antinuclear antibody (ANA >1:80) and/or anti-dsDNA (>30 IU/mL)]; AND
  • The patient has a history of at least 4 SLE related disease manifestations (SLE related disease manifestations include, but are not limited to: serositis malar rash, discoid rash, photosensitivity, oral ulcers, nonerosive arthritis, pleuritis/pericarditis, renal disorder [persistent proteinuria >0.5 grams/day or cellular casts], hematologic disorder [hemolytic anemia (with reticulocytosis), leucopenia, lymphopenia, positive finding of antiphospholipid antibodies, or anti-Sm antibodies]); AND
    • The patient is currently on a standard of care SLE treatment regimen comprised of at least one of the following: corticosteroids, antimalarials (e.g. hydroxychloroquine, chloroquine), nonsteroidal anti-inflammatory drugs (e.g. NSAIDS), aspirin, and/or immunosuppressives (e.g. azathioprine, methotrexate, cyclosporine, oral cyclophosphamide, or mycophenolate); OR
    • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to ALL the standard of care drug classes listed above

FDA Approved Indication(s); Compendia Recommended Indication(s); Ф Orphan Drug

  1. Renewal Criteria 1-4

Coverage may be renewed based upon the following criteria:

  • Patient continues to meet the universal and other indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in section III; AND
  • Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: severe infusion-related reactions, tumor lysis syndrome (TLS), severe mucocutaneous reactions, progressive multifocal leukoencephalopathy (PML), hepatitis B virus reactivation, serious infections (bacterial, fungal or viral), cardiovascular adverse reactions (e.g., ventricular fibrillation, myocardial infarction, cardiogenic shock, cardiac arrhythmias), renal toxicity, bowel obstruction and perforation, etc.; AND

Oncology Indications 1-5,23-25,34,44,50,62,80,94-98,102-104,129,130,102-104,128

  • Patient has not exceeded dosing or duration limits as defined in Sections I, II, and V; AND

Adult Acute Lymphoblastic Leukemia (ALL)

  • Treatment response or stabilization of disease as indicated by CBC, bone marrow cytogenic analysis, QPCR, or FISH

Pediatric B-Cell Acute Leukemia and Aggressive Mature B-Cell Lymphomas (induction or consolidation therapy)

  • Coverage may NOT be renewed

Pediatric Hodgkin Lymphoma

  • Coverage may NOT be renewed

Chronic Graft-Versus-Host Disease (cGVHD)

  • Coverage may NOT be renewed

Hematopoietic Cell Transplantation

  • Coverage may NOT be renewed

Management of Immunotherapy-Related Toxicities

  • Coverage for use in the treatment of myositis/myasthenia gravis/encephalitis may NOT be renewed
  • Coverage for use in bullous dermatitis: Patient has not exceeded a maximum of 18 months of therapy (4 total doses)

All Other Oncology Indications

  • Disease response with treatment as defined by stabilization of disease or decrease in size of tumor or tumor spread

Non-Oncology Indications 1-4

Rheumatoid Arthritis (RA)

  • Disease response as indicated by improvement in signs and symptoms compared to baseline such as the number of tender and swollen joint counts, reduction of C-reactive protein, improvement of patient global assessment, and/or an improvement on a disease activity scoring tool [e.g. an improvement on a composite scoring index such as Disease Activity Score-28 (DAS28) of 1.2 points or more or a ≥20% improvement on the American College of Rheumatology-20 (ACR20) criteria]; AND
  • Dose escalation (up to the maximum dose and frequency specified below) may occur upon clinical review on a case by case basis provided that the patient has:
    • Shown an initial response to therapy; AND
    • Received a minimum of one maintenance dose at the dose and interval specified below; AND
    • Responded to therapy with subsequent loss of response

Thrombocytopenic Purpura (ITP or Evans Syndrome)7-9

  • Disease response as indicated by the achievement and maintenance of a platelet count of at least 50 × 109/L as necessary to reduce the risk for bleeding

Thrombotic Thrombocytopenic Purpura (TTP)

  • Disease response as indicated by an increase in ADAMTS13 activity with a reduction in thrombotic risk

Multiple Sclerosis (MS) 147,151

  • Continuous monitoring of response to therapy indicates a beneficial response* [manifestations of MS disease activity include, but are not limited to, an increase in annualized relapse rate (ARR), development of new/worsening T2 hyperintensities or enhancing lesions on brain/spinal MRI, and progression of sustained impairment as evidenced by expanded disability status scale (EDSS), timed 25-foot walk (T25-FW), 9-hole peg test (9-HPT)]

*Note:

  • Inadequate response, in those who have been adherent and receiving therapy for sufficient time to realize the full treatment effect, is defined as ≥ 1 relapse, ≥ 2 unequivocally new MRI-detected lesions, or increased disability on examination over a one-year period.

Granulomatosis with Polyangiitis (GPA) (Wegener’s granulomatosis) and Microscopic Polyangiitis (MPA)

  • Disease response as indicated by disease control and improvement in signs and symptoms of condition compared to baseline; AND
  • Decreased frequency in the occurrence of major relapses (defined by the reappearance of clinical and/or laboratory signs of vasculitis activity that could lead to organ failure or damage, or could be life threatening)

Pemphigus Vulgaris 10,11,35,61

  • Patient is currently receiving tapering doses of corticosteroids or has discontinued use of corticosteroids; AND
    • Disease response as indicated by one of the following:
      • Complete epithelialization of lesions and improvement in signs and symptoms of condition compared to baseline
      • Patient has not developed new lesions and established lesions begin to heal
      • For Relapses ONLY: Patient previously achieved disease control; AND
        • Patient has the appearance of 3 or more new lesions a month that do not heal spontaneously within 1 week, or by the extension of established lesions

Autoimmune Hemolytic Anemia (AIHA).31,152

  • Disease response as indicated by improvement in signs and symptoms of anemia (e.g., dyspnea, fatigue, etc.); AND
  • Patient has had an improvement in laboratory values (e.g., hemoglobin, hematocrit, etc.), reduced transfusion needs, and/or reduced glucocorticoid use

Lupus Nephritis 115-117

  • Coverage may only be renewed in patients experiencing a disease relapse (e.g., increased serum creatinine, increase in protein urine excretion, decrease in eGFR, etc.)

Myasthenia Gravis (unrelated to immunotherapy-related toxicity) 118-120

  • Disease response as indicated by a decrease in the daily dose of corticosteroids and/or an improvement in signs and symptoms compared to baseline.

Complications of Transplanted Solid Organ (kidney, liver, lung, heart, pancreas) 133-138

  • Coverage may NOT be renewed.

NMOSD 90,91

  • Disease response as indicated by stabilization/improvement in any of the following:
    • Neurologic symptoms as evidenced by a decrease in acute relapses or improvement of stability
    • Reduced hospitalizations
    • Reduction/discontinuation in plasma exchange treatments
    • Reduction/discontinuation of corticosteroids without relapse
  1. Dosage/Administration 1-5,9,19,23-26,32,34,40,42,44,50,62,80,83-89,91,94-98,102-111,115-118,122-125,128-133,135-137,140,152,164,165

    Indication

    Dose

    CLL/SLL

    Initial Therapy

    375 mg/m² intravenously (IV) weekly for 8 doses; OR

    375 mg/m² IV cycle 1, then 500 mg/m² every 28 days cycles 2-6 (6 total doses); OR

    375 mg/m² IV cycle 1, followed by 500 mg/m² every 2 weeks for 4 doses, then 500 mg/m² every 28 days for 3 doses (8 total doses)

    Renewal Therapy

    375 mg/m² IV every 3 months; OR

    500 mg/ m² IV every 8 weeks

    Adult B-Cell Lymphomas, Primary Cutaneous B-Cell Lymphomas, Waldenström Macroglobulinemia, or Adult HL

    Initial Therapy

    375 mg/m2 IV once weekly for 4 – 8 doses in a 6 month period

    Renewal Therapy

    375 mg/m² IV once weekly for 4 doses per 6 month period; OR

    375 mg/ m² IV every 8 weeks

    Pediatric Aggressive Mature B-Cell Lymphomas

    Induction* [courses 1 and 2 (COPDAM1 and COPDAM2)]

    375 mg/m2 IV, two doses during each of the induction courses (Day -2 and Day 1).

    During the 1st induction course, prednisone is given as part of the chemotherapy course, and should be administered prior to rituximab. Rituximab will be given 48 hours after the first infusion of rituximab.

    Consolidation* [courses 1 and 2 (CYM/CYVE)]

    375 mg/m2 IV, one dose during each of the consolidation courses (Day 1)

    Relapsed/Refractory

    RCYVE – 375mg/m2 IV on day 1 of each 21-day cycle

    RICE – 375 mg/m2 IV on days 1 and 3 of courses 1 and 2, and on day 1 only of course 3 if needed.

    *Note: dosing and dosing schedules are highly variable and dependent on regimen used, please refer to NCCN and PI for additional protocols.

    Pediatric Mature B-Cell Acute Leukemia

    Induction* [courses 1 and 2 (COPDAM1 and COPDAM2)]

    375 mg/m2 IV, two doses during each of the induction courses (Day -2 and Day 1).

    During the 1st induction course, prednisone is given as part of the chemotherapy course, and should be administered prior to rituximab. Rituximab will be given 48 hours after the first infusion of rituximab.

    Consolidation* [courses 1 and 2 (CYM/CYVE)]

    375 mg/m2 IV, one dose during each of the consolidation courses (Day 1)

    *Note: dosing and dosing schedules are highly variable and dependent on regimen used, please refer to NCCN and PI for additional protocols.

    CNS Lymphoma

    Intravenous administration

    Initial Therapy: 375 mg/m2 IV once weekly for 4 – 8 doses in a 6 month period

    Renewal Therapy: 375 mg/m² IV once weekly for 4 doses per 6 month period; OR

    375 mg/m² IV every 8 weeks

    Intrathecal/Intraventricular administration

    10-40 mg weekly to every 3 weeks

    ALL

    375 mg/m2 IV up to twice weekly for a total of 16 to 18 infusions (e.g., induction [days 1 and 7], salvage reinduction when necessary [days 1 and 7], consolidation [4 infusions: blocks 1, 3, 4, and 6], late intensification [days 1 and 7], late consolidation [2 infusions: blocks 7 and 9], and maintenance [6 infusions])

    Hairy Cell Leukemia

    375 mg/m2 IV once weekly for 4 – 8 doses; OR

    375mg/m2 IV on days 1 and 15 every 28 days for 4 cycles, then 375mg/m2 IV every 4 weeks for 4 cycles (up to 8 total cycles)

    RA

    1,000 mg IV on days 1 and 15, repeated every 24 weeks. May repeat up to every 16 weeks following the previous infusion in patients requiring more frequent dosing based on clinical evaluation.

    Pemphigus

     Vulgaris

    Initiation

    1,000 mg IV on days 1 and 15; OR

    375 mg/m² IV weekly for 4 doses

    Maintenance

    500 mg IV at month 12 and repeat every 6 months thereafter or based on clinical evaluation

    Relapse

    1,000 mg IV upon relapse, resumption of glucocorticoids may be considered

    *Subsequent infusions (maintenance and relapse) should be no sooner than 16 weeks after the previous infusion.

    AIHA

    Warm-reactive disease

    375 mg/m² IV weekly for 4 doses in a 6 month period; OR

    1,000 mg IV on days 1 and 15

    Cold agglutinin disease

    375 mg/m² IV weekly for 4 doses in a 6 month period

    Thrombocytopenic Purpura or Thrombotic Thrombocytopenic Purpura (TTP)

    375 mg/m² IV weekly for 4 doses; OR

    1,000 mg IV on days 1 and 15

    Management of Immunotherapy-Related Toxicities

    Bullous Dermatitis

    1,000 mg IV every 2 weeks for 2 doses, then 500 mg IV at months 12 and 18 as needed

    Myositis
    375 mg/m² IV weekly for 4 doses

    Myasthenia Gravis

    375 mg/m² IV weekly for 4 doses; OR

    500 mg/m² IV every 2 weeks for 2 doses

    Encephalitis

    1,000 mg IV every 2 weeks for 2 doses; OR

    375 mg/m² IV weekly for 4 doses

    GPA (WG), MPA

    Induction (Pediatric and Adult)

    375 mg/m² IV weekly for 4 doses; OR

          • Adults: 1,000 mg IV on days 1 and 15; OR
          • Pediatric (up to a maximum of 1,000 mg per dose):
            • 575 mg/m² IV on days 1 and 15 (BSA ≤1.5m²)
            • 750 mg/m² IV on days 1 and 15 (BSA >1.5m²)

    Maintenance

          • Pediatric:
            • 250 mg/m² IV on days 1 and 15, then 250 mg/m² IV every 6 months thereafter based on clinical evaluation
          • Adult:
            • 500 mg IV on days 1 and 15, then 500 mg IV every 6 months thereafter based on clinical evaluation

    *Initial MAINTENANCE infusions should be no sooner than 16 weeks and no later than 24 weeks after the previous infusion if rituximab was used for initial induction therapy.

    *Initial MAINTENANCE infusions should be initiated within 4 weeks following disease control when initial induction occurred with other standard of care immunosuppressants.

    cGVHD

    375 mg/m² IV weekly for 4 doses, then 375 mg/m² IV monthly for 4 months

    -OR-

    375 mg/m² IV weekly for 4 doses (Note: A second course of 4 weekly doses may be administered 8 weeks after initial therapy for patients with lack of or incomplete response.)

    -OR-

    375 mg/m² IV weekly for 4 – 8 doses

    Hematopoietic Cell Transplantation

    Conditioning:

    375 mg/m² IV for 1 day before transplant, then 1000 mg/m² IV on days 1,8, and 15 after transplant

    Multiple Sclerosis

    1,000 mg IV on days 1 and 15, repeat every 6 months

    NMOSD

    1,000 mg IV once on days 1 and 15, repeat every 6 months

    -OR-

    375 mg/m2 once weekly for 4 weeks, repeat every 6 months

    Histiocytic Neoplasms Rosai-Dorfman Disease

    500 mg/m² IV every 1 – 2 weeks for 2 – 6 doses every 6 months

    Systemic Lupus Erythematosus

    375 mg/m2 IV once weekly for 4 doses

    Lupus Nephritis

    1,000 mg IV on days 1 and 15

    -OR-

    375 mg/m2 IV once weekly for 4 doses

    Myasthenia Gravis (unrelated to immunotherapy-related toxicity)

    1,000 mg IV on days 1 and 15, may repeat a full or partial course every 6 months

    -OR-

    375 mg/m2 IV once weekly for 4 doses, may repeat a full or partial course every 6 months

    Pediatric Hodgkin Lymphoma

    375 mg/m² IV on day 1 of every 2-3 week cycle for a total of 3 cycles

    Complications of transplanted solid organ (kidney, liver, lung, heart, pancreas)

          • Adults and pediatrics weighing ≥0.5 m2: 375 mg/m2 weekly for up to 4 doses
          • Pediatrics weighing <0.5 m2: 12.5 mg/kg weekly for up to 4 doses

    Abbreviations: COP = Cyclophosphamide, Oncovin (vincristine), Prednisone; COPDAM = Cyclophosphamide, Oncovin (vincristine), Prednisolone, Adriamycin (doxorubicin), Methotrexate; CYM = Cytarabine (Ara-C), Methotrexate; CYVE = Cytarabine (Ara-C), Vepesid (Etoposide,VP-16); RICE = Rituximab, Ifosfamide, Carboplatin, Etoposide (VP-16)

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

  1. Billing Code/Availability Information

HCPCS Code(s):

  • J9312 – Injection, rituximab, 10 mg; 1 billable unit = 10 mg (Rituxan IV only)
  • Q5115 – Injection, rituximab-abbs, biosimilar, (truxima), 10 mg; 1 billable unit = 10 mg
  • Q5119 – Injection, rituximab-pvvr, biosimilar, (ruxience), 10 mg; 1 billable unit = 10 mg
  • Q5123 – Injection, rituximab-arrx, biosimilar, (riabni), 10 mg; 1 billable unit = 10 mg

NDC(s):

  • Rituxan 100 mg/10 mL single-dose vial for injection: 50242-0051-xx
  • Rituxan 500 mg/50 mL single-dose vial for injection: 50242-0053-xx
  • Truxima 100 mg/10 mL single-dose vial for injection: 63459-0103-xx
  • Truxima 500 mg/50 mL single-dose vial for injection: 63459-0104-xx
  • Ruxience 100 mg/10 mL single-dose vial for injection: 00069-0238-xx
  • Ruxience 500 mg/50 mL single-dose vial for injection: 00069-0249-xx
  • Riabni 100 mg/10 mL single-dose vial for injection: 55513-0224-xx
  • Riabni 500 mg/50 mL single-dose vial for injection: 55513-0326-xx
  1. References
  1. Rituxan [package insert]. South San Francisco, CA; Genentech, Inc.; December 2021. Accessed November 2023.
  2. Truxima [package insert]. Incheon, Republic of Korea; Celltrion, Inc.; February 2022. Accessed November 2023.
  3. Ruxience [package insert]. New York, NY; Pfizer, Inc; October 2023. Accessed November 2023.
  4. Riabni [package insert]. Thousand Oaks, CA; Amgen, Inc.; June 2022. Accessed November2023.
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Appendix 1 – Covered Diagnosis Codes

ICD-10

Description

C79.32

Secondary malignant neoplasm of cerebral meninges

C81.00

Nodular lymphocyte predominant Hodgkin lymphoma, unspecified site

C81.01

Nodular lymphocyte predominant Hodgkin lymphoma, lymph nodes of head, face, and neck

C81.02

Nodular lymphocyte predominant Hodgkin lymphoma, intrathoracic lymph nodes

C81.03

Nodular lymphocyte predominant Hodgkin lymphoma, intra-abdominal lymph nodes

C81.04

Nodular lymphocyte predominant Hodgkin lymphoma, lymph nodes of axilla and upper limb

C81.05

Nodular lymphocyte predominant Hodgkin lymphoma, lymph nodes of inguinal region and lower limb

C81.06

Nodular lymphocyte predominant Hodgkin lymphoma, intrapelvic lymph nodes

C81.07

Nodular lymphocyte predominant Hodgkin lymphoma, spleen

C81.08

Nodular lymphocyte predominant Hodgkin lymphoma, lymph nodes of multiple sites

C81.09

Nodular lymphocyte predominant Hodgkin lymphoma, extranodal and solid organ sites

C81.19

Nodular sclerosis Hodgkin lymphoma, extranodal and solid organ sites

C81.29

Mixed cellularity Hodgkin lymphoma, extranodal and solid organ sites

C81.39

Lymphocyte depleted Hodgkin lymphoma, extranodal and solid organ sites

C81.49

Lymphocyte-rich Hodgkin lymphoma, extranodal and solid organ sites

C81.79

Other Hodgkin lymphoma, extranodal and solid organ sites

C81.99

Hodgkin lymphoma, unspecified, extranodal and solid organ sites

C82.00

Follicular lymphoma grade I, unspecified site

C82.01

Follicular lymphoma grade I, lymph nodes of head, face and neck

C82.02

Follicular lymphoma, grade I, intrathoracic lymph nodes

C82.03

Follicular lymphoma grade I, intra-abdominal lymph nodes

C82.04

Follicular lymphoma grade I, lymph nodes of axilla and upper limb

C82.05

Follicular lymphoma grade I, lymph nodes of inguinal regional and lower limb

C82.06

Follicular lymphoma grade I, intrapelvic lymph nodes

C82.07

Follicular lymphoma grade I, spleen

C82.08

Follicular lymphoma grade I, lymph nodes of multiple sites

C82.09

Follicular lymphoma grade I, extranodal and solid organ sites

C82.10

Follicular lymphoma grade II, unspecified site

C82.11

Follicular lymphoma grade II, lymph nodes of head, face and neck

C82.12

Follicular lymphoma, grade II, intrathoracic lymph nodes

C82.13

Follicular lymphoma grade II, intra-abdominal lymph nodes

C82.14

Follicular lymphoma grade II, lymph nodes of axilla and upper limb

C82.15

Follicular lymphoma grade II, lymph nodes of inguinal region and lower limb

C82.16

Follicular lymphoma grade II, intrapelvic lymph nodes

C82.17

Follicular lymphoma grade II, spleen

C82.18

Follicular lymphoma grade II, lymph nodes of multiple sites

C82.19

Follicular lymphoma grade II, extranodal and solid organ sites

C82.20

Follicular lymphoma grade III, unspecified, unspecified site

C82.21

Follicular lymphoma grade III, unspecified, lymph nodes of head, face and neck

C82.22

Follicular lymphoma, grade III, unspecified, intrathoracic lymph nodes

C82.23

Follicular lymphoma grade III, unspecified, intra-abdominal lymph nodes

C82.24

Follicular lymphoma grade III, unspecified, lymph nodes of axilla and upper limb

C82.25

Follicular lymphoma grade III, unspecified, lymph nodes of inguinal region and lower limb

C82.26

Follicular lymphoma grade III, unspecified, intrapelvic lymph nodes

C82.27

Follicular lymphoma grade III, unspecified, spleen

C82.28

Follicular lymphoma grade III, unspecified, lymph nodes of multiple sites

C82.29

Follicular lymphoma grade III, unspecified, extranodal and solid organ sites

C82.30

Follicular lymphoma grade IIIa, unspecified site

C82.31

Follicular lymphoma grade IIIa, lymph nodes of head, face and neck

C82.32

Follicular lymphoma, grade IIIa, intrathoracic lymph nodes

C82.33

Follicular lymphoma grade IIIa, intra-abdominal lymph nodes

C82.34

Follicular lymphoma grade IIIa, lymph nodes of axilla and upper limb

C82.35

Follicular lymphoma grade IIIa, lymph nodes of inguinal region and lower limb

C82.36

Follicular lymphoma grade IIIa, intrapelvic lymph nodes

C82.37

Follicular lymphoma grade IIIa, spleen

C82.38

Follicular lymphoma grade IIIa, lymph nodes of multiple sites

C82.39

Follicular lymphoma grade IIIa, extranodal and solid organ sites

C82.40

Follicular lymphoma grade IIIb, unspecified site

C82.41

Follicular lymphoma grade IIIb, lymph nodes of head, face and neck

C82.42

Follicular lymphoma, grade IIIb, intrathoracic lymph nodes

C82.43

Follicular lymphoma grade IIIb, intra-abdominal lymph nodes

C82.44

Follicular lymphoma grade IIIb, lymph nodes of axilla and upper limb

C82.45

Follicular lymphoma grade IIIb, lymph nodes of inguinal region and lower limb

C82.46

Follicular lymphoma grade IIIb, intrapelvic lymph nodes

C82.47

Follicular lymphoma grade IIIb, spleen

C82.48

Follicular lymphoma grade IIIb, lymph nodes of multiple sites

C82.49

Follicular lymphoma grade IIIb, extranodal and solid organ sites

C82.50

Diffuse follicle center lymphoma, unspecified site

C82.51

Diffuse follicle center lymphoma, lymph nodes of head, face and neck

C82.52

Diffuse follicle center lymphoma, intrathoracic lymph nodes

C82.53

Diffuse follicle center lymphoma, intra-abdominal lymph nodes

C82.54

Diffuse follicle center lymphoma, lymph nodes of axilla and upper limb

C82.55

Diffuse follicle center lymphoma, lymph nodes of inguinal region and lower limb

C82.56

Diffuse follicle center lymphoma, intrapelvic lymph nodes

C82.57

Diffuse follicle center lymphoma, spleen

C82.58

Diffuse follicle center lymphoma, lymph nodes of multiple sites

C82.59

Diffuse follicle center lymphoma, extranodal and solid organ sites

C82.60

Cutaneous follicle center lymphoma, unspecified site

C82.61

Cutaneous follicle center lymphoma, lymph nodes of head, face and neck

C82.62

Cutaneous follicle center lymphoma, intrathoracic lymph nodes

C82.63

Cutaneous follicle center lymphoma, intra-abdominal lymph nodes

C82.64

Cutaneous follicle center lymphoma, lymph nodes of axilla and upper limb

C82.65

Cutaneous follicle center lymphoma, lymph nodes of inguinal region and lower limb

C82.66

Cutaneous follicle center lymphoma, intrapelvic lymph nodes

C82.67

Cutaneous follicle center lymphoma, spleen

C82.68

Cutaneous follicle center lymphoma, lymph nodes of multiple sites

C82.69

Cutaneous follicle center lymphoma, extranodal and solid organ sites

C82.80

Other types of follicular lymphoma, unspecified site

C82.81

Other types of follicular lymphoma, lymph nodes of head, face and neck

C82.82

Other types of follicular lymphoma, intrathoracic lymph nodes

C82.83

Other types of follicular lymphoma, intra-abdominal lymph nodes

C82.84

Other types of follicular lymphoma, lymph nodes of axilla and upper limb

C82.85

Other types of follicular lymphoma, lymph nodes of inguinal region and lower limb

C82.86

Other types of follicular lymphoma, intrapelvic lymph nodes

C82.87

Other types of follicular lymphoma, spleen

C82.88

Other types of follicular lymphoma, lymph nodes of multiple sites

C82.89

Other types of follicular lymphoma, extranodal and solid organ sites

C82.90

Follicular lymphoma, unspecified, unspecified site

C82.91

Follicular lymphoma, unspecified, lymph nodes of head, face and neck

C82.92

Follicular lymphoma, unspecified, intrathoracic lymph nodes

C82.93

Follicular lymphoma, unspecified, intra-abdominal lymph nodes

C82.94

Follicular lymphoma, unspecified, lymph nodes of axilla and upper limb

C82.95

Follicular lymphoma, unspecified lymph nodes of inguinal region and lower limb

C82.96

Follicular lymphoma, unspecified, intrapelvic lymph nodes

C82.97

Follicular lymphoma, unspecified, spleen

C82.98

Follicular lymphoma, unspecified, lymph nodes of multiple sites

C82.99

Follicular lymphoma, unspecified, extranodal and solid organ sites

C83.00

Small cell B-cell lymphoma, unspecified site

C83.01

Small cell B-cell lymphoma, lymph nodes of head, face and neck

C83.02

Small cell B-cell lymphoma, intrathoracic lymph nodes

C83.03

Small cell B-cell lymphoma, intra-abdominal lymph nodes

C83.04

Small cell B-cell lymphoma, lymph nodes of axilla and upper limb

C83.05

Small cell B-cell lymphoma, lymph nodes of inguinal region and lower limb

C83.06

Small cell B-cell lymphoma, intrapelvic lymph nodes

C83.07

Small cell B-cell lymphoma, spleen

C83.08

Small cell B-cell lymphoma, lymph nodes of multiple sites

C83.09

Small cell B-cell lymphoma, extranodal and solid organ sites

C83.10

Mantle cell lymphoma, unspecified site

C83.11

Mantle cell lymphoma, lymph nodes of head, face and neck

C83.12

Mantle cell lymphoma, intrathoracic lymph nodes

C83.13

Mantle cell lymphoma, intra-abdominal lymph nodes

C83.14

Mantle cell lymphoma, lymph nodes of axilla and upper limb

C83.15

Mantle cell lymphoma, lymph nodes of inguinal region and lower limb

C83.16

Mantle cell lymphoma, intrapelvic lymph nodes

C83.17

Mantle cell lymphoma, spleen

C83.18

Mantle cell lymphoma, lymph nodes of multiple sites

C83.19

Mantle cell lymphoma, extranodal and solid organ sites

C83.30

Diffuse large B-cell lymphoma unspecified site

C83.31

Diffuse large B-cell lymphoma, lymph nodes of head, face, and neck

C83.32

Diffuse large B-cell lymphoma intrathoracic lymph nodes

C83.33

Diffuse large B-cell lymphoma intra-abdominal lymph nodes

C83.34

Diffuse large B-cell lymphoma lymph nodes of axilla and upper limb

C83.35

Diffuse large B-cell lymphoma, lymph nodes of inguinal region and lower limb

C83.36

Diffuse large B-cell lymphoma intrapelvic lymph nodes

C83.37

Diffuse large B-cell lymphoma, spleen

C83.38

Diffuse large B-cell lymphoma lymph nodes of multiple sites

C83.39

Diffuse large B-cell lymphoma extranodal and solid organ sites

C83.50

Lymphoblastic (diffuse) lymphoma, unspecified site

C83.51

Lymphoblastic (diffuse) lymphoma, lymph nodes of head, face, and neck

C83.52

Lymphoblastic (diffuse) lymphoma, intrathoracic lymph nodes

C83.53

Lymphoblastic (diffuse) lymphoma, intra-abdominal lymph nodes

C83.54

Lymphoblastic (diffuse) lymphoma, lymph nodes of axilla and upper limb

C83.55

Lymphoblastic (diffuse) lymphoma, lymph nodes of inguinal region and lower limb

C83.56

Lymphoblastic (diffuse) lymphoma, intrapelvic lymph nodes

C83.57

Lymphoblastic (diffuse) lymphoma, spleen

C83.58

Lymphoblastic (diffuse) lymphoma, lymph nodes of multiple sites

C83.59

Lymphoblastic (diffuse) lymphoma, extranodal and solid organ sites

C83.70

Burkitt lymphoma, unspecified site

C83.71

Burkitt lymphoma, lymph nodes of head, face, and neck

C83.72

Burkitt lymphoma, intrathoracic lymph nodes

C83.73

Burkitt lymphoma, intra-abdominal lymph nodes

C83.74

Burkitt lymphoma, lymph nodes of axilla and upper limb

C83.75

Burkitt lymphoma, lymph nodes of inguinal region and lower limb

C83.76

Burkitt lymphoma, intrapelvic lymph nodes

C83.77

Burkitt lymphoma, spleen

C83.78

Burkitt lymphoma, lymph nodes of multiple sites

C83.79

Burkitt lymphoma, extranodal and solid organ sites

C83.80

Other non-follicular lymphoma, unspecified site

C83.81

Other non-follicular lymphoma, lymph nodes of head, face and neck

C83.82

Other non-follicular lymphoma, intrathoracic lymph nodes

C83.83

Other non-follicular lymphoma, intra-abdominal lymph nodes

C83.84

Other non-follicular lymphoma, lymph nodes of axilla and upper limb

C83.85

Other non-follicular lymphoma, lymph nodes of inguinal region and lower limb

C83.86

Other non-follicular lymphoma, intrapelvic lymph nodes

C83.87

Other non-follicular lymphoma, spleen

C83.88

Other non-follicular lymphoma, lymph nodes of multiple sites

C83.89

Other non-follicular lymphoma, extranodal and solid organ sites

C83.90

Non-follicular (diffuse) lymphoma, unspecified site

C83.91

Non-follicular (diffuse) lymphoma, unspecified lymph nodes of head, face, and neck

C83.92

Non-follicular (diffuse) lymphoma, unspecified intrathoracic lymph nodes

C83.93

Non-follicular (diffuse) lymphoma, unspecified intra-abdominal lymph nodes

C83.94

Non-follicular (diffuse) lymphoma, unspecified lymph nodes of axilla and upper limb

C83.95

Non-follicular (diffuse) lymphoma, unspecified lymph nodes of inguinal region and lower limb

C83.96

Non-follicular (diffuse) lymphoma, unspecified intrapelvic lymph nodes

C83.97

Non-follicular (diffuse) lymphoma, unspecified spleen

C83.98

Non-follicular (diffuse) lymphoma, unspecified lymph nodes of multiple sites

C83.99

Non-follicular (diffuse) lymphoma, unspecified extranodal and solid organ sites

C84.09

Mycosis fungoides, extranodal and solid organ sites

C84.19

Sézary disease, extranodal and solid organ sites

C84.49

Peripheral T-cell lymphoma, not classified, extranodal and solid organ sites

C84.69

Anaplastic large cell lymphoma, ALK-positive, extranodal and solid organ sites

C84.79

Anaplastic large cell lymphoma, ALK-negative, extranodal and solid organ sites

C84.99

Mature T/NK-cell lymphomas, unspecified, extranodal and solid organ sites

C84.A9

Cutaneous T-cell lymphoma, unspecified, extranodal and solid organ sites

C84.Z9

Other mature T/NK-cell lymphomas, extranodal and solid organ sites

C85.10

Unspecified B-cell lymphoma, unspecified site

C85.11

Unspecified B-cell lymphoma, lymph nodes of head, face, and neck

C85.12

Unspecified B-cell lymphoma, intrathoracic lymph nodes

C85.13

Unspecified B-cell lymphoma, intra-abdominal lymph nodes

C85.14

Unspecified B-cell lymphoma, lymph nodes of axilla and upper limb

C85.15

Unspecified B-cell lymphoma, lymph nodes of inguinal region and lower limb

C85.16

Unspecified B-cell lymphoma, intrapelvic lymph nodes

C85.17

Unspecified B-cell lymphoma, spleen

C85.18

Unspecified B-cell lymphoma, lymph nodes of multiple sites

C85.19

Unspecified B-cell lymphoma, extranodal and solid organ sites

C85.20

Mediastinal (thymic) large B-cell lymphoma, unspecified site

C85.21

Mediastinal (thymic) large B-cell lymphoma, lymph nodes of head, face and neck

C85.22

Mediastinal (thymic) large B-cell lymphoma, intrathoracic lymph nodes

C85.23

Mediastinal (thymic) large B-cell lymphoma, intra-abdominal lymph nodes

C85.24

Mediastinal (thymic) large B-cell lymphoma, lymph nodes of axilla and upper limb

C85.25

Mediastinal (thymic) large B-cell lymphoma, lymph nodes of inguinal region and lower limb

C85.26

Mediastinal (thymic) large B-cell lymphoma, intrapelvic lymph nodes

C85.27

Mediastinal (thymic) large B-cell lymphoma, spleen

C85.28

Mediastinal (thymic) large B-cell lymphoma, lymph nodes of multiple sites

C85.29

Mediastinal (thymic) large B-cell lymphoma, extranodal and solid organ sites

C85.80

Other specified types of non-Hodgkin lymphoma, unspecified site

C85.81

Other specified types of non-Hodgkin lymphoma, lymph nodes of head, face and neck

C85.82

Other specified types of non-Hodgkin lymphoma, intrathoracic lymph nodes

C85.83

Other specified types of non-Hodgkin lymphoma, intra-abdominal lymph nodes

C85.84

Other specified types of non-Hodgkin lymphoma, lymph nodes of axilla and upper limb

C85.85

Other specified types of non-Hodgkin lymphoma, lymph nodes of inguinal region of lower limb

C85.86

Other specified types of non-Hodgkin lymphoma, intrapelvic lymph nodes

C85.87

Other specified types of non-Hodgkin lymphoma, spleen

C85.88

Other specified types of non-Hodgkin lymphoma, lymph nodes of multiple sites

C85.89

Other specified types of non-Hodgkin lymphoma, extranodal and solid organ sites

C85.99

Non-Hodgkin lymphoma, unspecified, extranodal and solid organ sites

C88.0

Waldenström macroglobulinemia

C88.4

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-lymphoma)

C91.00

Acute lymphoblastic leukemia not having achieved remission

C91.01

Acute lymphoblastic leukemia, in remission

C91.02

Acute lymphoblastic leukemia, in relapse

C91.10

Chronic lymphocytic leukemia of B-cell type not having achieved remission

C91.12

Chronic lymphocytic leukemia of B-cell type in relapse

C91.40

Hairy cell leukemia not having achieved remission

C91.42

Hairy cell leukemia, in relapse

D47.Z1

Post-transplant lymphoproliferative disorder (PTLD)

D47.Z2

Other neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue-Castleman disease

D59.10

Autoimmune hemolytic anemia, unspecified

D59.11

Warm autoimmune hemolytic anemia

D59.12

Cold autoimmune hemolytic anemia

D59.13

Mixed type autoimmune hemolytic anemia

D59.19

Other autoimmune hemolytic anemia

D69.3

Immune thrombocytopenic purpura

D69.41

Evans Syndrome

D69.42

Congenital and hereditary thrombocytopenia purpura

D69.49

Other primary thrombocytopenia

D76.3

Other histiocytosis syndromes

D89.811

Chronic graft-versus-host disease

D89.812

Acute on chronic graft-versus-host disease

D89.813

Graft-versus-host disease unspecified

G04.81

Other encephalitis and encephalomyelitis

G04.89

Other myelitis

G04.90

Encephalitis and encephalomyelitis, unspecified

G35

Multiple sclerosis

G36.0

Neuromyelitis optica [Devic]

G70.0

Myasthenia gravis

G70.00

Myasthenia gravis without (acute) exacerbation

G70.01

Myasthenia gravis with (acute) exacerbation

L10.0

Pemphigus vulgaris

L13.8

Other specified bullous disorders

L13.9

Bullous disorder, unspecified

M05.10

Rheumatoid lung disease with rheumatoid arthritis of unspecified site

M05.111

Rheumatoid lung disease with rheumatoid arthritis of right shoulder

M05.112

Rheumatoid lung disease with rheumatoid arthritis of left shoulder

M05.119

Rheumatoid lung disease with rheumatoid arthritis of unspecified shoulder

M05.121

Rheumatoid lung disease with rheumatoid arthritis of right elbow

M05.122

Rheumatoid lung disease with rheumatoid arthritis of left elbow

M05.129

Rheumatoid lung disease with rheumatoid arthritis of unspecified elbow

M05.131

Rheumatoid lung disease with rheumatoid arthritis of right wrist

M05.132

Rheumatoid lung disease with rheumatoid arthritis of left wrist

M05.139

Rheumatoid lung disease with rheumatoid arthritis of unspecified wrist

M05.141

Rheumatoid lung disease with rheumatoid arthritis of right hand

M05.142

Rheumatoid lung disease with rheumatoid arthritis of left hand

M05.149

Rheumatoid lung disease with rheumatoid arthritis of unspecified hand

M05.151

Rheumatoid lung disease with rheumatoid arthritis of right hip

M05.152

Rheumatoid lung disease with rheumatoid arthritis of left hip

M05.159

Rheumatoid lung disease with rheumatoid arthritis of unspecified hip

M05.161

Rheumatoid lung disease with rheumatoid arthritis of right knee

M05.162

Rheumatoid lung disease with rheumatoid arthritis of left knee

M05.169

Rheumatoid lung disease with rheumatoid arthritis of unspecified knee

M05.171

Rheumatoid lung disease with rheumatoid arthritis of right ankle and foot

M05.172

Rheumatoid lung disease with rheumatoid arthritis of left ankle and foot

M05.179

Rheumatoid lung disease with rheumatoid arthritis of unspecified ankle and foot

M05.19

Rheumatoid lung disease with rheumatoid arthritis of multiple sites

M05.20

Rheumatoid vasculitis with rheumatoid arthritis of unspecified site

M05.211

Rheumatoid vasculitis with rheumatoid arthritis of right shoulder

M05.212

Rheumatoid vasculitis with rheumatoid arthritis of left shoulder

M05.219

Rheumatoid vasculitis with rheumatoid arthritis of unspecified shoulder

M05.221

Rheumatoid vasculitis with rheumatoid arthritis of right elbow

M05.222

Rheumatoid vasculitis with rheumatoid arthritis of left elbow

M05.229

Rheumatoid vasculitis with rheumatoid arthritis of unspecified elbow

M05.231

Rheumatoid vasculitis with rheumatoid arthritis of right wrist

M05.232

Rheumatoid vasculitis with rheumatoid arthritis of left wrist

M05.239

Rheumatoid vasculitis with rheumatoid arthritis of unspecified wrist

M05.241

Rheumatoid vasculitis with rheumatoid arthritis of right hand

M05.242

Rheumatoid vasculitis with rheumatoid arthritis of left hand

M05.249

Rheumatoid vasculitis with rheumatoid arthritis of unspecified hand

M05.251

Rheumatoid vasculitis with rheumatoid arthritis of right hip

M05.252

Rheumatoid vasculitis with rheumatoid arthritis of left hip

M05.259

Rheumatoid vasculitis with rheumatoid arthritis of unspecified hip

M05.261

Rheumatoid vasculitis with rheumatoid arthritis of right knee

M05.262

Rheumatoid vasculitis with rheumatoid arthritis of left knee

M05.269

Rheumatoid vasculitis with rheumatoid arthritis of unspecified knee

M05.271

Rheumatoid vasculitis with rheumatoid arthritis of right ankle and foot

M05.272

Rheumatoid vasculitis with rheumatoid arthritis of left ankle and foot

M05.279

Rheumatoid vasculitis with rheumatoid arthritis of unspecified ankle and foot

M05.29

Rheumatoid vasculitis with rheumatoid arthritis of multiple sites

M05.30

Rheumatoid heart disease with rheumatoid arthritis of unspecified site

M05.311

Rheumatoid heart disease with rheumatoid arthritis of right shoulder

M05.312

Rheumatoid heart disease with rheumatoid arthritis of left shoulder

M05.319

Rheumatoid heart disease with rheumatoid arthritis of unspecified shoulder

M05.321

Rheumatoid heart disease with rheumatoid arthritis of right elbow

M05.322

Rheumatoid heart disease with rheumatoid arthritis of left elbow

M05.329

Rheumatoid heart disease with rheumatoid arthritis of unspecified elbow

M05.331

Rheumatoid heart disease with rheumatoid arthritis of right wrist

M05.332

Rheumatoid heart disease with rheumatoid arthritis of left wrist

M05.339

Rheumatoid heart disease with rheumatoid arthritis of unspecified wrist

M05.341

Rheumatoid heart disease with rheumatoid arthritis of right hand

M05.342

Rheumatoid heart disease with rheumatoid arthritis of left hand

M05.349

Rheumatoid heart disease with rheumatoid arthritis of unspecified hand

M05.351

Rheumatoid heart disease with rheumatoid arthritis of right hip

M05.352

Rheumatoid heart disease with rheumatoid arthritis of left hip

M05.359

Rheumatoid heart disease with rheumatoid arthritis of unspecified hip

M05.361

Rheumatoid heart disease with rheumatoid arthritis of right knee

M05.362

Rheumatoid heart disease with rheumatoid arthritis of left knee

M05.369

Rheumatoid heart disease with rheumatoid arthritis of unspecified knee

M05.371

Rheumatoid heart disease with rheumatoid arthritis of right ankle and foot

M05.372

Rheumatoid heart disease with rheumatoid arthritis of left ankle and foot

M05.379

Rheumatoid heart disease with rheumatoid arthritis of unspecified ankle and foot

M05.39

Rheumatoid heart disease with rheumatoid arthritis of multiple sites

M05.40

Rheumatoid myopathy with rheumatoid arthritis of unspecified site

M05.411

Rheumatoid myopathy with rheumatoid arthritis of right shoulder

M05.412

Rheumatoid myopathy with rheumatoid arthritis of left shoulder

M05.419

Rheumatoid myopathy with rheumatoid arthritis of unspecified shoulder

M05.421

Rheumatoid myopathy with rheumatoid arthritis of right elbow

M05.422

Rheumatoid myopathy with rheumatoid arthritis of left elbow

M05.429

Rheumatoid myopathy with rheumatoid arthritis of unspecified elbow

M05.431

Rheumatoid myopathy with rheumatoid arthritis of right wrist

M05.432

Rheumatoid myopathy with rheumatoid arthritis of left wrist

M05.439

Rheumatoid myopathy with rheumatoid arthritis of unspecified wrist

M05.441

Rheumatoid myopathy with rheumatoid arthritis of right hand

M05.442

Rheumatoid myopathy with rheumatoid arthritis of left hand

M05.449

Rheumatoid myopathy with rheumatoid arthritis of unspecified hand

M05.451

Rheumatoid myopathy with rheumatoid arthritis of right hip

M05.452

Rheumatoid myopathy with rheumatoid arthritis of left hip

M05.459

Rheumatoid myopathy with rheumatoid arthritis of unspecified hip

M05.461

Rheumatoid myopathy with rheumatoid arthritis of right knee

M05.462

Rheumatoid myopathy with rheumatoid arthritis of left knee

M05.469

Rheumatoid myopathy with rheumatoid arthritis of unspecified knee

M05.471

Rheumatoid myopathy with rheumatoid arthritis of right ankle and foot

M05.472

Rheumatoid myopathy with rheumatoid arthritis of left ankle and foot

M05.479

Rheumatoid myopathy with rheumatoid arthritis of unspecified ankle and foot

M05.49

Rheumatoid myopathy with rheumatoid arthritis of multiple sites

M05.50

Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified site

M05.511

Rheumatoid polyneuropathy with rheumatoid arthritis of right shoulder

M05.512

Rheumatoid polyneuropathy with rheumatoid arthritis of left shoulder

M05.519

Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified shoulder

M05.521

Rheumatoid polyneuropathy with rheumatoid arthritis of right elbow

M05.522

Rheumatoid polyneuropathy with rheumatoid arthritis of left elbow

M05.529

Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified elbow

M05.531

Rheumatoid polyneuropathy with rheumatoid arthritis of right wrist

M05.532

Rheumatoid polyneuropathy with rheumatoid arthritis of left wrist

M05.539

Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified wrist

M05.541

Rheumatoid polyneuropathy with rheumatoid arthritis of right hand

M05.542

Rheumatoid polyneuropathy with rheumatoid arthritis of left hand

M05.549

Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified hand

M05.551

Rheumatoid polyneuropathy with rheumatoid arthritis of right hip

M05.552

Rheumatoid polyneuropathy with rheumatoid arthritis of left hip

M05.559

Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified hip

M05.561

Rheumatoid polyneuropathy with rheumatoid arthritis of right knee

M05.562

Rheumatoid polyneuropathy with rheumatoid arthritis of left knee

M05.569

Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified knee

M05.571

Rheumatoid polyneuropathy with rheumatoid arthritis of right ankle and foot

M05.572

Rheumatoid polyneuropathy with rheumatoid arthritis of left ankle and foot

M05.579

Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified ankle and foot

M05.59

Rheumatoid polyneuropathy with rheumatoid arthritis of multiple sites

M05.60

Rheumatoid arthritis of unspecified site with involvement of other organs and systems

M05.611

Rheumatoid arthritis of right shoulder with involvement of other organs and systems

M05.612

Rheumatoid arthritis of left shoulder with involvement of other organs and systems

M05.619

Rheumatoid arthritis of unspecified shoulder with involvement of other organs and systems

M05.621

Rheumatoid arthritis of right elbow with involvement of other organs and systems

M05.622

Rheumatoid arthritis of left elbow with involvement of other organs and systems

M05.629

Rheumatoid arthritis of unspecified elbow with involvement of other organs and systems

M05.631

Rheumatoid arthritis of right wrist with involvement of other organs and systems

M05.632

Rheumatoid arthritis of left wrist with involvement of other organs and systems

M05.639

Rheumatoid arthritis of unspecified wrist with involvement of other organs and systems

M05.641

Rheumatoid arthritis of right hand with involvement of other organs and systems

M05.642

Rheumatoid arthritis of left hand with involvement of other organs and systems

M05.649

Rheumatoid arthritis of unspecified hand with involvement of other organs and systems

M05.651

Rheumatoid arthritis of right hip with involvement of other organs and systems

M05.652

Rheumatoid arthritis of left hip with involvement of other organs and systems

M05.659

Rheumatoid arthritis of unspecified hip with involvement of other organs and systems

M05.661

Rheumatoid arthritis of right knee with involvement of other organs and systems

M05.662

Rheumatoid arthritis of left knee with involvement of other organs and systems

M05.669

Rheumatoid arthritis of unspecified knee with involvement of other organs and systems

M05.671

Rheumatoid arthritis of right ankle and foot with involvement of other organs and systems

M05.672

Rheumatoid arthritis of left ankle and foot with involvement of other organs and systems

M05.679

Rheumatoid arthritis of unspecified ankle and foot with involvement of other organs and systems

M05.69

Rheumatoid arthritis of multiple sites with involvement of other organs and systems

M05.7A

Rheumatoid arthritis with rheumatoid factor of other specified site without organ or systems involvement

M05.711

Rheumatoid arthritis with rheumatoid factor of right shoulder without organ or systems involvement

M05.712

Rheumatoid arthritis with rheumatoid factor of left shoulder without organ or systems involvement

M05.719

Rheumatoid arthritis with rheumatoid factor of unspecified shoulder without organ or systems involvement

M05.721

Rheumatoid arthritis with rheumatoid factor of right elbow without organ or systems involvement

M05.722

Rheumatoid arthritis with rheumatoid factor of left elbow without organ or systems involvement

M05.729

Rheumatoid arthritis with rheumatoid factor of unspecified elbow without organ or systems involvement

M05.731

Rheumatoid arthritis with rheumatoid factor of right wrist without organ or systems involvement

M05.732

Rheumatoid arthritis with rheumatoid factor of left wrist without organ or systems involvement

M05.739

Rheumatoid arthritis with rheumatoid factor of unspecified wrist without organ or systems involvement

M05.741

Rheumatoid arthritis with rheumatoid factor of right hand without organ or systems involvement

M05.742

Rheumatoid arthritis with rheumatoid factor of left hand without organ or systems involvement

M05.749

Rheumatoid arthritis with rheumatoid factor of unspecified hand without organ or systems involvement

M05.751

Rheumatoid arthritis with rheumatoid factor of right hip without organ or systems involvement

M05.752

Rheumatoid arthritis with rheumatoid factor of left hip without organ or systems involvement

M05.759

Rheumatoid arthritis with rheumatoid factor of unspecified hip without organ or systems involvement

M05.761

Rheumatoid arthritis with rheumatoid factor of right knee without organ or systems involvement

M05.762

Rheumatoid arthritis with rheumatoid factor of left knee without organ or systems involvement

M05.769

Rheumatoid arthritis with rheumatoid factor of unspecified knee without organ or systems involvement

M05.771

Rheumatoid arthritis with rheumatoid factor of right ankle and foot without organ or systems involvement

M05.772

Rheumatoid arthritis with rheumatoid factor of left ankle and foot without organ or systems involvement

M05.779

Rheumatoid arthritis with rheumatoid factor of unspecified ankle and foot without organ or systems involvement

M05.79

Rheumatoid arthritis with rheumatoid factor of multiple sites without organ or systems involvement

M05.8A

Other rheumatoid arthritis with rheumatoid factor of other specified site

M05.811

Other rheumatoid arthritis with rheumatoid factor of right shoulder

M05.812

Other rheumatoid arthritis with rheumatoid factor of left shoulder

M05.819

Other rheumatoid arthritis with rheumatoid factor of unspecified shoulder

M05.821

Other rheumatoid arthritis with rheumatoid factor of right elbow

M05.822

Other rheumatoid arthritis with rheumatoid factor of left elbow

M05.829

Other rheumatoid arthritis with rheumatoid factor of unspecified elbow

M05.831

Other rheumatoid arthritis with rheumatoid factor of right wrist

M05.832

Other rheumatoid arthritis with rheumatoid factor of left wrist

M05.839

Other rheumatoid arthritis with rheumatoid factor of unspecified wrist

M05.841

Other rheumatoid arthritis with rheumatoid factor of right hand

M05.842

Other rheumatoid arthritis with rheumatoid factor of left hand

M05.849

Other rheumatoid arthritis with rheumatoid factor of unspecified hand

M05.851

Other rheumatoid arthritis with rheumatoid factor of right hip

M05.852

Other rheumatoid arthritis with rheumatoid factor of left hip

M05.859

Other rheumatoid arthritis with rheumatoid factor of unspecified hip

M05.861

Other rheumatoid arthritis with rheumatoid factor of right knee

M05.862

Other rheumatoid arthritis with rheumatoid factor of left knee

M05.869

Other rheumatoid arthritis with rheumatoid factor of unspecified knee

M05.871

Other rheumatoid arthritis with rheumatoid factor of right ankle and foot

M05.872

Other rheumatoid arthritis with rheumatoid factor of left ankle and foot

M05.879

Other rheumatoid arthritis with rheumatoid factor of unspecified ankle and foot

M05.89

Other rheumatoid arthritis with rheumatoid factor of multiple sites

M05.9

Rheumatoid arthritis with rheumatoid factor, unspecified

M06.0A

Rheumatoid arthritis without rheumatoid factor, other specified site

M06.011

Rheumatoid arthritis without rheumatoid factor, right shoulder

M06.012

Rheumatoid arthritis without rheumatoid factor, left shoulder

M06.019

Rheumatoid arthritis without rheumatoid factor, unspecified shoulder

M06.021

Rheumatoid arthritis without rheumatoid factor, right elbow

M06.022

Rheumatoid arthritis without rheumatoid factor, left elbow

M06.029

Rheumatoid arthritis without rheumatoid factor, unspecified elbow

M06.031

Rheumatoid arthritis without rheumatoid factor, right wrist

M06.032

Rheumatoid arthritis without rheumatoid factor, left wrist

M06.039

Rheumatoid arthritis without rheumatoid factor, unspecified wrist

M06.041

Rheumatoid arthritis without rheumatoid factor, right hand

M06.042

Rheumatoid arthritis without rheumatoid factor, left hand

M06.049

Rheumatoid arthritis without rheumatoid factor, unspecified hand

M06.051

Rheumatoid arthritis without rheumatoid factor, right hip

M06.052

Rheumatoid arthritis without rheumatoid factor, left hip

M06.059

Rheumatoid arthritis without rheumatoid factor, unspecified hip

M06.061

Rheumatoid arthritis without rheumatoid factor, right knee

M06.062

Rheumatoid arthritis without rheumatoid factor, left knee

M06.069

Rheumatoid arthritis without rheumatoid factor, unspecified knee

M06.071

Rheumatoid arthritis without rheumatoid factor, right ankle and foot

M06.072

Rheumatoid arthritis without rheumatoid factor, left ankle and foot

M06.079

Rheumatoid arthritis without rheumatoid factor, unspecified ankle and foot

M06.08

Rheumatoid arthritis without rheumatoid factor, vertebrae

M06.09

Rheumatoid arthritis without rheumatoid factor, multiple sites

M06.8A

Other specified rheumatoid arthritis, other specified site

M06.811

Other specified rheumatoid arthritis, right shoulder

M06.812

Other specified rheumatoid arthritis, left shoulder

M06.819

Other specified rheumatoid arthritis, unspecified shoulder

M06.821

Other specified rheumatoid arthritis, right elbow

M06.822

Other specified rheumatoid arthritis, left elbow

M06.829

Other specified rheumatoid arthritis, unspecified elbow

M06.831

Other specified rheumatoid arthritis, right wrist

M06.832

Other specified rheumatoid arthritis, left wrist

M06.839

Other specified rheumatoid arthritis, unspecified wrist

M06.841

Other specified rheumatoid arthritis, right hand

M06.842

Other specified rheumatoid arthritis, left hand

M06.849

Other specified rheumatoid arthritis, unspecified hand

M06.851

Other specified rheumatoid arthritis, right hip

M06.852

Other specified rheumatoid arthritis, left hip

M06.859

Other specified rheumatoid arthritis, unspecified hip

M06.861

Other specified rheumatoid arthritis, right knee

M06.862

Other specified rheumatoid arthritis, left knee

M06.869

Other specified rheumatoid arthritis, unspecified knee

M06.871

Other specified rheumatoid arthritis, right ankle and foot

M06.872

Other specified rheumatoid arthritis, left ankle and foot

M06.879

Other specified rheumatoid arthritis, unspecified ankle and foot

M06.88

Other specified rheumatoid arthritis, vertebrae

M06.89

Other specified rheumatoid arthritis, multiple sites

M06.9

Rheumatoid arthritis, unspecified

M31.10

Thrombotic microangiopathy, unspecified

M31.30

Wegener’s granulomatosis without renal involvement

M31.31

Wegener’s granulomatosis with renal involvement

M31.7

Microscopic polyangiitis

M32.10

Systemic lupus erythematosus organ or system involvement unspecified

M32.11

Endocarditis in systemic lupus erythematosus

M32.12

Pericarditis in systemic lupus erythematosus

M32.13

Lung involvement in systemic lupus erythematosus

M32.14

Glomerular disease in systemic lupus erythematosus

M32.15

Tubulo-interstitial nephropathy in systemic lupus erythematosus

M32.19

Other organ or system involvement in systemic lupus erythematosus

M32.8

Other forms of systemic lupus erythematosus

M32.9

Systemic lupus erythematosus, unspecified

M60.80

Other myositis, unspecified site

M60.811

Other myositis, right shoulder

M60.812

Other myositis, left shoulder

M60.819

Other myositis, unspecified shoulder

M60.821

Other myositis, right upper arm

M60.822

Other myositis, left upper arm

M60.829

Other myositis, unspecified upper arm

M60.831

Other myositis, right forearm

M60.832

Other myositis, left forearm

M60.839

Other myositis, unspecified forearm

M60.841

Other myositis, right hand

M60.842

Other myositis, left hand

M60.849

Other myositis, unspecified hand

M60.851

Other myositis, right thigh

M60.852

Other myositis, left thigh

M60.859

Other myositis, unspecified thigh

M60.861

Other myositis, right lower leg

M60.862

Other myositis, left lower leg

M60.869

Other myositis, unspecified lower leg

M60.871

Other myositis, right ankle and foot

M60.872

Other myositis, left ankle and foot

M60.879

Other myositis, unspecified ankle and foot

M60.88

Other myositis, other site

M60.89

Other myositis, multiple sites

M79.10

Myalgia, unspecified site

M79.11

Myalgia of mastication muscle

M79.12

Myalgia of auxiliary muscles, head and neck

M79.18

Myalgia, other site

T86.09

Other complications of bone marrow transplant

Z85.71

Personal history of Hodgkin lymphoma

Z85.72

Personal history of non-Hodgkin lymphomas

Z85.79

Personal history of other malignant neoplasms of lymphoid, hematopoietic and related tissues

Z94.81

Bone marrow transplant status

Z94.89

Other transplanted organ and tissue status

Z94.9

Transplanted organ and tissue status, unspecified

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determination (NCD), Local Coverage Articles (LCAs), and Local Coverage Determinations (LCDs) may exist and compliance with these policies is required where applicable. They can be found at: https://www.cms.gov/medicare-coverage-database/search.aspx. Additional indications may be covered at the discretion of the health plan.

Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCD/LCA):

Jurisdiction(s): 6, K

NCD/LCD/LCA Document (s): A59101

https://www.cms.gov/medicare-coverage-database/new-search/search-results.aspx?keyword=a59101&areaId=all&docType=NCA%2CCAL%2CNCD%2CMEDCAC%2CTA%2CMCD%2C6%2C3%2C5%2C1%2CF%2CP

Jurisdiction(s): 5,8

NCD/LCD/LCA Document (s): A55639

https://www.cms.gov/medicare-coverage-database/new-search/search-results.aspx?keyword=a55639&areaId=all&docType=NCA%2CCAL%2CNCD%2CMEDCAC%2CTA%2CMCD%2C6%2C3%2C5%2C1%2CF%2CP

Jurisdiction(s): J, M

NCD/LCD/LCA Document (s): A56380

https://www.cms.gov/medicare-coverage-database/new-search/search-results.aspx?keyword=a56380&areaId=all&docType=NCA%2CCAL%2CNCD%2CMEDCAC%2CTA%2CMCD%2C6%2C3%2C5%2C1%2CF%2CP

Jurisdiction(s): 15

NCD/LCD/LCA Document (s): A57160

https://www.cms.gov/medicare-coverage-database/new-search/search-results.aspx?keyword=a57160&areaId=all&docType=NCA%2CCAL%2CNCD%2CMEDCAC%2CTA%2CMCD%2C6%2C3%2C5%2C1%2CF%2CP

Jurisdiction(s): 15

NCD/LCD/LCA Document (s): A58582

https://www.cms.gov/medicare-coverage-database/new-search/search-results.aspx?keyword=a58582&areaId=all&docType=NCA%2CCAL%2CNCD%2CMEDCAC%2CTA%2CMCD%2C6%2C3%2C5%2C1%2CF%2CP

Medicare Part B Administrative Contractor (MAC) Jurisdictions

Jurisdiction

Applicable State/US Territory

Contractor

E (1)

CA, HI, NV, AS, GU, CNMI

Noridian Healthcare Solutions, LLC

F (2 & 3)

AK, WA, OR, ID, ND, SD, MT, WY, UT, AZ

Noridian Healthcare Solutions, LLC

5

KS, NE, IA, MO

Wisconsin Physicians Service Insurance Corp (WPS)

6

MN, WI, IL

National Government Services, Inc. (NGS)

H (4 & 7)

LA, AR, MS, TX, OK, CO, NM

Novitas Solutions, Inc.

8

MI, IN

Wisconsin Physicians Service Insurance Corp (WPS)

N (9)

FL, PR, VI

First Coast Service Options, Inc.

J (10)

TN, GA, AL

Palmetto GBA, LLC

M (11)

NC, SC, WV, VA (excluding below)

Palmetto GBA, LLC

L (12)

DE, MD, PA, NJ, DC (includes Arlington & Fairfax counties and the city of Alexandria in VA)

Novitas Solutions, Inc.

K (13 & 14)

NY, CT, MA, RI, VT, ME, NH

National Government Services, Inc. (NGS)

15

KY, OH

CGS Administrators, LLC

 

 

 

 

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