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Plasma Exchange (Plasmapheresis)

Policy Number: MP-100

Latest Review Date: July 2023

Category: Therapy                                                       

POLICY:

Plasma exchange (plasmapheresis) may be considered medically necessary for ANY of the following conditions:

Autoimmune

  • Severe multiple manifestation of mixed cryoglobulinemia (MC) such as cryoglobulinemic nephropathy, skin ulcers, sensory motor neuropathy, and widespread vasculitis in combination with immunosuppressive treatment;
  • Catastrophic antiphospholipid syndrome

Hematologic

  • ABO incompatible hematopoietic progenitor cell transplantation;
  • Hyperviscosity syndromes associated with multiple myeloma or Waldenström’s  macroglobulinemia;
  • Idiopathic thrombocytopenic purpura in emergency situations;
  • Thrombotic thrombocytopenic purpura (TTP);
  • Atypical hemolytic-uremic syndrome;
  • Post transfusion purpura;
  • HELLP syndrome of pregnancy (a severe form of preeclampsia, characterized by hemolysis [H], elevated liver enzymes [EL], and low platelet [LP] counts);
  • Myeloma with acute renal failure

Neurological

  • Acute inflammatory demyelinating polyneuropathy (Guillain-Barre’ syndrome [GBS]; severity grade 1-2 within two weeks of onset; and children less than 10 years old with severe GBS);
  • Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP);
  • Multiple sclerosis with acute fulminant CNS demyelination;
  • Myasthenia gravis in crisis or as part of preoperative preparation;
  • Paraproteinemia polyneuropathy; IgA, IgG;
  • Neuromyelitis optica
  • N-methyl-d-aspartate receptor antibody encephalitis;
  • Progressive multifocal leukoencephalopathy associated with natalizumab;

Renal

  • Anti-glomerular basement membrane disease (Goodpasture’s syndrome);
  • ANCA-associated vasculitis [e.g., Wegener’s granulomatosis, also known as granulomatosis with polyangiitis (GPA)] with associated renal failure;
  • Dense deposit disease with factor H deficiency and/or elevated C3 Nephritic factor

Transplantation

  • ABO incompatible solid organ transplantation:
  • Kidney;
  • heart (infants);
  • Renal transplantation: antibody mediated rejection; HLA [human leukocyte antigen] desensitization;
  • Focal segmental glomerulosclerosis after renal transplant

Plasma exchange (plasmapheresis) is considered investigational in all other conditions, including, but not limited, to the following:

  • ABO incompatible solid organ transplant; liver
  • Acute disseminated encephalomyelitis
  • Acute inflammatory demyelinating polyneuropathy (Guillain-Barre syndrome) in children less than 10 years old with mild or moderate forms
  • Acute liver failure
  • Amyotrophic lateral sclerosis (ALS)
  • ANCA-associated vasculitis [e.g., Wegener’s granulomatosis, also known as granulomatosis with polyangiitis (GPA)] without associated renal failure
  • Aplastic anemia
  • Asthma
  • Autoimmune hemolytic anemia; warm autoimmune hemolytic anemia; cold agglutinin disease
  • Chronic fatigue syndrome
  • Coagulation factor inhibitors
  • Cryoglobulinemia; except for severe mixed cryoglobulinemia; as noted above
  • Dermatomyositis and polymyositis
  • Focal segmental glomerulosclerosis (other than after renal transplant)
  • Heart transplant rejection treatment
  • Hemolytic uremic syndrome (HUS); typical (diarrheal-related)
  • Hyperviscosity syndromes with renal failure (other than associated with multiple myeloma or Waldenström’s macroglobulinemia).
  • Idiopathic thrombocytopenic purpura; refractory or non-refractory
  • Inclusion body myositis
  • Lambert-Eaton myasthenic syndrome
  • Macular Degeneration (Age Related)
  • Multiple sclerosis with chronic progressive or relapsing remitting course
  • Mushroom poisoning
  • Myasthenia gravis with anti-MuSK antibodies
  • Obsessive compulsive disorder
  • Overdose and poisoning (other than mushroom poisoning)
  • Paraneoplastic syndromes
  • Paraproteinemia polyneuropathy; IgM
  • Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS)
  • Pemphigus vulgaris
  • Phytanic acid storage disease (Refsum’s disease)
  • POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes)
  • Psoriasis
  • Red cell alloimmunization in pregnancy
  • Regional enteritis (Crohn’s disease)
  • Rheumatoid arthritis
  • Scleroderma (systemic sclerosis)
  • Sepsis
  • Stiff-man syndrome
  • Sydenham’s chorea
  • Systemic lupus erythematosus (including SLE nephritis)
  • Thyrotoxicosis

Individual case consideration for coverage of plasma exchange (plasmapheresis) will be given for patients with acute, life-threatening complications of chronic autoimmune diseases, such as rheumatoid arthritis and systemic lupus erythematosus. However, in these situations, treatment goal and duration of treatment with PE need to be clearly established prior to its initiation to ensure that short-term treatment of the acute complication does not evolve to a chronic use of PE with uncertain benefit.

DESCRIPTION OF PROCEDURE OR SERVICE:

Plasma exchange (PE) is a procedure in which the plasma is isolated, then discarded and replaced with a substitution fluid such as albumin. Plasma exchange is a nonspecific therapy, because the entire plasma is discarded. PE has been used in a wide variety of acute and chronic conditions, as well as in the setting of solid organ transplantation.

The terms therapeutic apheresis, plasmapheresis, and plasma exchange (PE) are often used interchangeably, but when properly used denote different procedures. The American Society for Apheresis (ASFA) definitions for these procedures are as follows:

  • Apheresis: A procedure in which blood of the patient or donor is passed through a medical device, which separates out one or more components of blood and returns remainder with or without extracorporeal treatment or replacement of the separated component.
  • Plasmapheresis: A procedure in which blood of a patient or the donor is passed through a medical device which separates out plasma from the other components of blood and the plasma is removed (i.e., less than 15% of total plasma volume) without the use of replacement solution.
  • Plasma exchange: A therapeutic procedure in which blood of the patient is passed through a medical device, which separates out plasma from other components of blood, the plasma, is removed and replaced with a replacement solution such as colloid solution (e.g., albumin and/ or plasma) or a combination of crystalloid/colloid solution.

This policy addresses only plasma exchange as a therapeutic apheresis procedure.

The rationale for PE is because circulating substances, such as toxins or autoantibodies, can accumulate in the plasma. In addition, it is hypothesized that removal of these factors can be therapeutic in certain situations. PE is essentially a symptomatic therapy, since it does not remove the source of the pathogenic factors. Therefore, the success of PE will depend on whether the pathogenic substances are accessible through the circulation and whether PE can adequately address their rate of production and transfer to the plasma component. For example, PE can rapidly reduce levels of serum autoantibodies; however, through a feedback mechanism, this rapid reduction may lead to a rebound overproduction of the same antibodies. This rebound production of antibodies is thought to render the replicating pathogenic clone of lymphocytes more vulnerable to cytotoxic drugs; therefore, PE is sometimes used in conjunction with cyclophosphamide.

Applications of PE can be broadly subdivided into two general categories: 1) acute self-limited diseases, in which PE is used to acutely lower the circulating pathogenic substance; and 2) chronic diseases, in which there is ongoing production of pathogenic autoantibodies. Because PE does not address underlying pathology, and, due to the phenomenon of rebound antibody production, its use in chronic diseases has been more controversial than in acute self-limited diseases.

In addition, plasmapheresis has been used in the setting of solid organ transplantation. It has been used as a technique to desensitize high-risk patients prior to transplant and as a treatment of antibody-mediated rejection reaction (AMR) occurring after transplant. Prior to transplant, plasmapheresis has been most commonly used to desensitize patients receiving an ABO mismatched kidney, often in combination with a splenectomy. As a treatment of AMR, plasmapheresis is often used in combination with intravenous immunoglobulin (IVIG) or anti-CD20 therapy (i.e., rituximab).

KEY POINTS:

This policy has been updated regularly with searches of the PubMed database. The most recent literature update was performed through July 7, 2023.

Summary of Evidence

In conclusion, due to data from published studies and/or clinical support, plasma exchange meets medical criteria for coverage for selected conditions. For conditions other than those selected, plasma exchange is considered investigational.

Practice Guidelines and Position Statements

National Comprehensive Cancer Network

In the current National Comprehensive Cancer Network guidelines on multiple myeloma (v.5.2022), use of plasmapheresis to improve renal function is a category 2B recommendation. Plasmapheresis should also be used as adjunctive therapy for hyperviscosity.

American Academy of Neurology

In 2011, the American Academy of Neurology issued evidence-based guidelines on plasmapheresis for the treatment of neurologic disorders. The primary conclusions, based on the evidence review, are provided in Table 1.

Table 1. Guidelines on Use Plasmapheresis to Treat Neurologic Disorders

Recommendation

Conclusion

Acute inflammatory demyelinating polyneuropathy/Guillain-Barré syndrome

Established effective

Chronic inflammatory demyelinating polyneuropathy, short-term treatment

Established effective

Relapses in multiple sclerosis

Probably effective

Fulminant demyelinating central nervous system disease

Possibly effective

Chronic or secondary progressive multiple sclerosis

Established ineffective

Myasthenia gravis

Insufficient evidence

Sydenham chorea

Insufficient evidence

Acute obsessive-compulsive disorder and tics in PANDAS

Insufficient evidence

PANDAS: pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections.

 

In 2003, the American Academy of Neurology published a practice parameter on Guillain-Barré syndrome (GBS). The following are the key findings: (1) treatment with plasma exchange (PE) or intravenous immunoglobulin hastens recovery from GBS; (2) combining the 2 treatments is not beneficial; and (3) steroid treatment given alone is not beneficial. The American Academy of Neurology’s recommendations are:

  • PE is recommended for adults with GBS who are nonambulant and who seek treatment within 4 weeks of the onset of neuropathic symptoms;
  • PE should be considered for ambulant patients examined within 2 weeks of the onset of neuropathic symptoms);
  • PE is a treatment option for children with severe GBS.

American Society for Apheresis

In 2016, the American Society for Apheresis updated its guidelines on the use of therapeutic apheresis (Seventh Special Issue). Previously, the guidelines had been updated in 2013 (Sixth Special Issue). The following is a description of the Society categories (see Table 2), 2013 recommendations (see Table 3), and new indications added in 2016 (see Table 4).

Table 2. American Society for Apheresis Categories

Category

Description

I

Diseases for which TA is accepted as first-line treatment, either as a primary standalone treatment or in conjunction with other treatments. Note that this designation need not imply that TA is mandatory in all cases.

II

Diseases for which TA is accepted as second-line treatment, either as a standalone treatment or in conjunction with other treatments.

III

Diseases for which the optimum role of TA is not established and treatment decisions on an individual basis are recommended.

IV

Disorders for which published evidence suggests or demonstrates that TA is ineffective or harmful.

TA: therapeutic apheresis.

Table 3. American Society for Apheresis 2013 Key Recommendations

Disease Group/Name/Condition

2013 Category

Autoimmune

 

Catastrophic antiphospholipid syndrome

II

Cryoglobulinemia

I

Pemphigus vulgaris

III

Systemic lupus erythematosus

 

Manifestations other than nephritis

NC

Severe

II

Nephritis

IV

Hematologic

 

ABO-incompatible hematopoietic progenitor cell transplantation

II

Aplastic anemia

III

Pure red blood cell aplasia

III

Autoimmune hemolytic anemia:

 

Warm autoimmune hemolytic anemia

III

Cold agglutinin disease

II

Coagulation factor inhibitors

IV

Hyperviscosity in monoclonal gammopathies

I

Idiopathic thrombocytopenic purpura

NC

Refractory immunoadsorption

NC

Refractory or nonrefractory

NC

Myeloma and acute renal failure (in 2010 and 2013 myeloma cast nephropathy)

 

Post-transfusion purpura

III

Red blood cell alloimmunization in pregnancy

III

Thrombotic thrombocytopenic purpura

I

Metabolic

 

Acute liver failure

III

Sepsis

III

Thyrotoxicosis

III

Neurologic

 

Acute disseminated encephalomyelitis

II

AIDP (Guillain-Barré syndrome)

I

AIDP, post IVIg

III

Chronic inflammatory demyelinating polyradiculoneuropathy

I

Lambert-Eaton myasthenic syndrome

II

Multiple sclerosis

 

Acute CNS inflammatory demyelinating disease

II

Devic syndrome

NC

Chronic progressive

III

Myasthenia gravis

 

In 2013, moderate-severe

I

In 2013, pre-thymectomy

I

Paraneoplastic neurologic syndromes

III

Paraproteinemic polyneuropathies

 

IgG/IgA

I

IgM

I

Multiple myeloma

III

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections; SC

 

PANDAS (2007, severe)

I

SC (2007, severe)

I

Rasmussen encephalitis

III

Stiff person syndrome

III

Neuromyelitis optica spectrum disorders

 

 Acute

II

 Maintenance

III

Renal

 

ANCA-associated rapidly progressive glomerulonephritis (Wegener granulomatosis)

 

Dialysis dependence

I

Dialysis independence

III

Antiglomerular basement membrane disease (Goodpasture’s syndrome)

 

DAH

I

Dialysis dependence and no DAH

III

Dialysis independence

I

Focal segmental glomerulosclerosis

 

Primary

NC

Secondary

NC

Recurrent

I

HUS; thrombotic microangiopathy; transplant-associated microangiopathy

 

Idiopathic HUS

NC

Transplant-associated microangiopathy

NC

Diarrhea-associated pediatric

NC

Atypical HUS due to complement factor H

I

Diarrhea-associated HUS or typical HUS

 

In 2013, Shiga toxin-associated

IV

Streptococcus pneumoniae associated

III

Renal transplantation: antibody-mediated rejection; HLA desensitization

 

Antibody-mediated rejection

I

HLA desensitization

II

Desensitization, living-donor, positive cross-match due to donor-specific HLA antibody

I

High PRA: cadaveric donor

III

Rheumatic

 

Scleroderma (progressive systemic sclerosis)

III

Transplantation

 

ABO-incompatible solid organ transplantation

 

Kidney

 

In 2013, desensitization, living-donor

I

Humeral rejection

II

Heart (infants)

NC

Liver (2010 perioperative)

 

In 2013, desensitization living-donor

I

Desensitization, deceased-donor

II

Humeral rejection

III

Heart transplant rejection

 

Treatment

NC

ABO: A, B, and O blood types; AIDP: acute inflammatory demyelinating polyneuropathy; ANCA: antineutrophil cytoplasmic antibody; CNS: central nervous system; DAH: diffuse alveolar hemorrhage; HLA: human leukocyte antigen; HUS: hemolytic uremic syndrome; Ig: immunoglobulin; IVIG: intravenous immunoglobulin; NC: not categorized; PANDAS: pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection; PRA: Panel Reactive Antibody; SC: Sydenham chorea.

Table 4. American Society for Apheresis New Indications in 2016

Disease Group/Name/Condition

2016 Category

Atopic (neuro-) dermatitis (atopic eczema), recalcitrant

III

Cardiac neonatal lupus

III

Complex regional pain syndrome

III

Erythropoietic porphyria, liver disease

III

Hashimoto encephalopathy: steroid-responsive encephalopathy associated with autoimmune thyroiditis

II

HELLP syndromea

 

Postpartum

III

Antepartum

IV

Hematopoietic cell transplantation, human leukocyte antigen desensitization

III

Hemophagocytic lymphohistiocytosis; hemophagocytic syndrome; macrophage activating syndrome

III

N-methyl-d-aspartate receptor antibody encephalitis

I

Prevention of Rhesus D alloimmunization after red blood cell exposure

III

Progressive multifocal leukoencephalopathy associated with natalizumab

I

Pruritus due to hepatobiliary diseases

III

Thrombotic microangiopathy, coagulation mediated

III

Vasculitis

 

HBV-PAN

II

Idiopathic PAN

IV

EGPA

III

Behçet disease

III

EGPA: eosinophilic granulomatosis with polyangiitis; HBV: hepatitis B virus; PAN: polyarteritis nodosa.

a A severe form of preeclampsia, characterized by hemolysis, elevated liver enzymes, and low platelet counts.

U.S. Preventative Services Task Force Recommendations

Not applicable

KEY WORDS:

Plasma exchange, plasmapheresis, apheresis, therapeutic apheresis, therapeutic plasma exchange, Rheo, Rheopheresis, focal glomerulosclerosis, FGS, focal segmental glomerulosclerosis, FSGS, Neuromyelitis optica, NMO, Devic syndrome

APPROVED BY GOVERNING BODIES:

The U.S. Food and Drug Administration has a compliance program to ensure that source plasma, source leukocytes, and therapeutic exchange plasma for further manufacture into products for human use are safe, pure, potent, and appropriately labeled. The compliance program covers products intended for use in both injectable drug products (e.g., immune globulin, albumin) and noninjectable products (e.g., in vitro devices such as blood bank reagents). Product Code for Therapeutic Exchange Plasma (TEP): 57DI-65.

BENEFIT APPLICATION:

Coverage is subject to member’s specific benefits.  Group-specific policy will supersede this policy when applicable.

ITS: Home Policy provisions apply

FEP: Special benefit consideration may apply.  Refer to member’s benefit plan.  

CODING: 

CPT code:

36514

Therapeutic apheresis; for plasma pheresis

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POLICY HISTORY:

TEC Assessment, November 1998

Medical Policy Group, March 2003 (2)

Medical Policy Administration Committee, March 2003

Available for comment April 1-May 16, 2003

Medical Policy Group, March 2005 (1)

Medical Policy Administration Committee, July 2005 (2)

Available for comment July 28-September 10, 2005

Medical Policy Group, January 2006 (2)

Medical Policy Administration Committee, February 2006

Available for comment March 4-April 17, 2006

Medical Policy Group, September 2006 (4)

Medical Policy Administration Committee, September 2006

Available for comment September 22-November 5, 2006

Medical Policy Group, September 2008 (1)

Medical Policy Administration Committee, October 2008

Available for comment October 4-November 17, 2008

Medical Policy Group, June 2009 (1)

Medical Policy Administration Committee, July 2009

Available for comment July 2-August 15, 2009

Medical Policy Group, September 2009 (2)

Medical Policy Administration Committee, October 2009

Available for comment October 3-November 17, 2009

Medical Policy Group, February 2010 (1): Updated policy for covered and non-covered conditions, reference list

Medical Policy Administration Committee, April 2010

Available for comment April 7-May 21, 2010

Medical Policy Group, January 2012 (3): Updated Key Points and References

Medical Policy Panel, May 2013

Medical Policy Group, May 2013 (1): Update to Policy with added coverage for CAPS, myeloma with renal failure, dense deposit disease with factor H and/or C3 Nephritic factor and focal segmental glomerulosclerosis after renal transplant. Serum creatinine threshold removed from ANCA-associated vasculitis criteria; updated Key Points and References

Available for comment May 22 through July 5, 2013

Medical Policy Panel, May 2014

Medical Policy Group, June 2014 (1): Update to Key Points and References; no change to policy statement

Medical Policy Panel, May 2015

Medical Policy Group, June 2015 (3): 2015 Updates to Key Points & References; no change in policy statement (only verbiage updates added clarifying some abbreviations of conditions listed.

Medical Policy Panel, September 2017

Medical Policy Group, October 2017 (7): Updates to Key Points and References. Policy Statement- added coverage for “N-methyl-d-aspartate receptor antibody encephalitis and Progressive multifocal leukoencephalopathy associated with natalizumab.”

Medical Policy Group, November 2020 (3): Update to Key Points and References; no change to policy statement.

Medical Policy Group, August 2021 (3): Updates to Key Points and References. Policy statement updated to remove “not medically necessary”, no other changes to policy statement or intent.

A peer reviewed literature analysis was completed and no new information was identified that would alter the coverage statement of this policy.

Medical Policy Group, August 2022 (3): 2022 Updates to Key Points, Practice Guidelines and Position Statements, and References. A peer reviewed literature analysis was completed and no new information was identified that would alter the coverage statement of this policy.

Medical Policy Group, July 2023 (11): 2023 Updates to Key Points, Benefit Application, and References. A peer reviewed literature analysis was completed and no new information was identified that would alter the coverage statement of this policy.

This medical policy is not an authorization, certification, explanation of benefits, or a contract. Eligibility and benefits are determined on a case-by-case basis according to the terms of the member’s plan in effect as of the date services are rendered. All medical policies are based on (i) research of current medical literature and (ii) review of common medical practices in the treatment and diagnosis of disease as of the date hereof. Physicians and other providers are solely responsible for all aspects of medical care and treatment, including the type, quality, and levels of care and treatment.

This policy is intended to be used for adjudication of claims (including pre-admission certification, pre-determinations, and pre-procedure review) in Blue Cross and Blue Shield’s administration of plan contracts.

The plan does not approve or deny procedures, services, testing, or equipment for our members. Our decisions concern coverage only. The decision of whether or not to have a certain test, treatment or procedure is one made between the physician and his/her patient. The plan administers benefits based on the member’s contract and corporate medical policies. Physicians should always exercise their best medical judgment in providing the care they feel is most appropriate for their patients. Needed care should not be delayed or refused because of a coverage determination.

As a general rule, benefits are payable under health plans only in cases of medical necessity and only if services or supplies are not investigational, provided the customer group contracts have such coverage.

The following Association Technology Evaluation Criteria must be met for a service/supply to be considered for coverage:

1. The technology must have final approval from the appropriate government regulatory bodies;

2. The scientific evidence must permit conclusions concerning the effect of the technology on health outcomes;

3. The technology must improve the net health outcome;

4. The technology must be as beneficial as any established alternatives;

5. The improvement must be attainable outside the investigational setting.

Medical Necessity means that health care services (e.g., procedures, treatments, supplies, devices, equipment, facilities or drugs) that a physician, exercising prudent clinical judgment, would provide to a patient for the purpose of preventing, evaluating, diagnosing or treating an illness, injury or disease or its symptoms, and that are:

1. In accordance with generally accepted standards of medical practice; and

2. Clinically appropriate in terms of type, frequency, extent, site and duration and considered effective for the patient’s illness, injury or disease; and

3. Not primarily for the convenience of the patient, physician or other health care provider; and

4. Not more costly than an alternative service or sequence of services at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of that patient’s illness, injury or disease.