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02-33000-17

Original Effective Date: 04/15/01

Reviewed: 06/22/17

Revised: 07/15/17

Subject: Apheresis, Plasmapheresis and Plasma Exchange

THIS MEDICAL COVERAGE GUIDELINE IS NOT AN AUTHORIZATION, CERTIFICATION, EXPLANATION OF BENEFITS, OR A GUARANTEE OF PAYMENT, NOR DOES IT SUBSTITUTE FOR OR CONSTITUTE MEDICAL ADVICE. ALL MEDICAL DECISIONS ARE SOLELY THE RESPONSIBILITY OF THE PATIENT AND PHYSICIAN. BENEFITS ARE DETERMINED BY THE GROUP CONTRACT, MEMBER BENEFIT BOOKLET, AND/OR INDIVIDUAL SUBSCRIBER CERTIFICATE IN EFFECT AT THE TIME SERVICES WERE RENDERED. THIS MEDICAL COVERAGE GUIDELINE APPLIES TO ALL LINES OF BUSINESS UNLESS OTHERWISE NOTED IN THE PROGRAM EXCEPTIONS SECTION.

           
Position Statement Billing/Coding Reimbursement Program Exceptions Definitions Related Guidelines
           
Other References Updates    
           

DESCRIPTION:

Apheresis, Plasmapheresis, and Plasma Exchange

The terms apheresis, plasmapheresis, and plasma exchange (PE) are often used interchangeably. The American Society for Apheresis (ASFA) defines these procedures as follows:

Apheresis: A procedure in which blood of the individual or donor is passed through a medical device which separates out one or more components of blood and returns the remainder with or without extracorporeal treatment or replacement of the separated component.

Plasmapheresis: A procedure in which blood of a individual or the donor is passed through a medical device which separates out plasma from the other components of blood and the plasma is removed (ie, <15% of total plasma volume) without the use of replacement solution.

Plasma exchange (PE): A therapeutic procedure in which blood of the individual 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 (eg, albumin and/ or plasma) or a combination of crystalloid/colloid solution.

The rationale for these procedures is based on the fact that circulating substances, such as toxins or autoantibodies, can accumulate in the plasma. Also, it is hypothesized that removal of these factors can be therapeutic in certain situations. PE is essentially a symptomatic therapy, because 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 their rate of production and transfer to the plasma component can be adequately addressed by PE. 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 apheresis, plasmapheresis, and plasma exchange can be broadly subdivided into 2 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 candidates before transplant and also as a treatment of antibody mediated rejection (AMR) reaction occurring after transplant. Before transplant, plasmapheresis has been most commonly used to desensitize candidates 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-CD-20 therapy (ie, Rituxan).

Low-Density Lipid (LDL) Apheresis

Lipid apheresis is used for disorders with marked hyperlipidemia, primarily familial hypercholesterolemia (FH). A dominantly inherited disorder, FH results froms a mutation in the gene that encodes for the specific cell surface receptor responsible for LDL uptake by the cells. The heterozygous form affects about 1 in 500 people. The number of LDL receptors is halved in this condition, resulting in serum low density lipoprotein cholesterol (LDL-C) levels that are approximately 2 to 3 times levels considered acceptable (ie, >300 mg/dL). Affected male patients typically develop coronary heart disease in their thirties and forties, while women develop the disease in their fifties. Depending on the patient, heterozygous FH may or may not respond adequately to lipid-lowering drugs.

The American Heart Association has indicated that adults with heterozygous familial hypercholesterolemia (FH) should be treated with available pharmacotherapy with an initial goal of reducing low-density lipoprotein cholesterol (LDL-C) by at least 50%, usually with a statin. This treatment can be followed by achieving an LDL-C of less than 100 mg/dL (absent coronary artery disease [CAD] or other major risk factors]) or 70 mg/dL (presence of CAD or other major risk factors). The following approach for pharmacotherapy is suggested:

• High-intensity statin therapy to target >50% LDL-C reduction, such as rosuvastatin or atorvastatin

• If the patient is adherent and LDL-C is above the target goal after 3 months, consider adding ezetimibe

• If the patient is adherent and LDL-C is above the target goal after 3 months, consider adding a PCSK9 inhibitor or colesevelam (or other bile acid sequestrant or niacin)

• If the patient is adherent and LDL-C is above the target goal after 3 months, proceed to complex therapy combination such as a 4-drug combination plus LDL apheresis

Low-density lipid (LDL) apheresis is a technique by which cholesterol is removed from the blood by first isolating the plasma, then selectively removing the low density lipoproteins from the plasma by either immunoadsorption, heparin-induced extracorporeal LDL precipitation, or dextran sulfate adsorption. The plasma is then returned to the individual.

LDL apheresis has been developed as a technique to treat those with familial hypercholesterolemia (FH). FH is a dominantly inherited disorder involving a mutation of the gene that encodes for the specific cell surface receptor responsible for LDL uptake by the cells. The heterozygous form affects about 1 in 500 people.The number of LDL receptors is halved in this condition, resulting in serum low-density lipoprotein cholesterol (LDL-C) levels that are approximately 2 to 3 times levels that are considered acceptable (ie, >300 mg/dL). Affected men typically develop coronary heart disease in their thirties and forties, while affected women develop coronary heart disease in their fifties. Depending on the individual, heterozygous FH may or may not respond adequately to lipid-lowering drugs.

Homozygous hypercholesterolemia is rare, only occurring in 1 in 1 million subjects. Serum levels of LDLC may be elevated 6-fold (>500 mg/dL), due to the total lack of functioning LDL receptors. Homozygotes may develop severe aortic stenosis and coronary heart disease by age 20 years. These individuals typically do not adequately respond to drug or diet modification therapy. In the past, those with homozygous FH may have been treated with plasma exchange, but the advent of LDL apheresis provides a more targeted approach by permitting selective removal of LDL from the plasma.

Therapeutic apheresis with selective HDL delipidation and plasma reinfusion is a procedure in which plasma is removed from the body by apheresis, processed through a delipidation device and then returned to the body. The delipidation procedure selectively removes cholesterol from HDL, converting the major alpha HDL to pre-beta-like HDL. The plasma with pre-beta-like HDL is then reinfused to the individual. The pre-beta-like HDL is a form of HDL that enhances cholesterol transport to the liver and is thought to reduce atherosclerosis development and burden.

POSITION STATEMENT:

Apheresis, Plasmapheresis, and Plasma Exchange (36511-36515)

Apheresis, plasmapheresis, and plasma exchange meet the definition of medical necessity for the following conditions:

Autoimmune

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

• Catastrophic antiphospholipid syndrome (CAPS)

Hematologic

• ABO incompatible hematopoietic progenitor cell transplantation

• Hyperviscosity syndromes associated with multiple myeloma or Waldenström 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

Familial hypercholesterolemia

Neurologic

• Acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome [GBS]; severity grade 1-2 within 2 weeks of onset severity grade 3-5 within 4 weeks of onset; and children younger than 10 years old with severe GBS)

• Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

• Multiple sclerosis (MS), with acute fulminant central nervous system (CNS) demyelination

• Myasthenia gravis in crisis or as part of preoperative preparation

• Paraproteinemia polyneuropathy; IgA, IgG

Renal

• Antiglomerular basement membrane disease (Goodpasture syndrome)

• ANCA (antineutrophil cytoplasmic antibody)‒associated vasculitis [eg, Wegener granulomatosis (also known as granulomatosis with polyangitis [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 and HLA [human leukocyte antigen] desensitization)

• Focal segmental glomerulosclerosis after renal transplant

Apheresis, plasmapheresis, and plasma exchange is considered experimental or investigational for all other conditions, including but not limited to the conditions listed below. There is insufficient evidence in the published medical literature to permit conclusions on safety, efficacy and long-term outcomes.

• ABO-incompatible solid organ transplant; liver

• Acute disseminated encephalomyelitis

• Acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome) in children younger than 10 years old with mild or moderate forms

• Acute liver failure

• Amyotrophic lateral sclerosis

• ANCA [(antineutrophil cytoplasmic antibody)‒associated rapidly progressive glomerulonephritis (Wegener granulomatosis or GPA without 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)

• Idiopathic thrombocytopenic purpura; refractory or nonrefractory

• Inclusion body myositis

• Lambert-Eaton myasthenic syndrome

• Multiple sclerosis with chronic progressive or relapsing remitting course

• Mushroom poisoning

• Myasthenia gravis with anti-MuSK antibodies

• 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 disease)

• POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes)

• Psoriasis

• Red blood cell alloimmunization in pregnancy

• Rheumatoid arthritis

• Sepsis

• Scleroderma (systemic sclerosis)

• Stiff person syndrome

• Sydenham chorea (SC)

• Systemic lupus erythematosus (including SLE nephritis)

• Thyrotoxicosis

• Hyperviscosity syndromes with renal failure (other than associated with multiple myeloma or Waldenström macroglobulinemia)

Low-density Lipid (LDL) Apheresis (36516, S2120)

Low-density lipid (LDL) apheresis meets the definition of medical necessity for homozygous familial hypercholesterolemia as an alternative to plasmapheresis.

Low-density lipid (LDL) apheresis as an alternative to plasmapheresis meets the definition of medical necessity for heterozygous familial hypercholesterolemia (FH), when ALL of the following are met:

• Failed diet therapy and maximum tolerated combination drug therapy AND meets ONE of the following FDA-approved indications:

• Functional hypercholesterolemic heterozygotes with LDL ≥300 mg/dL, OR

• Functional hypercholesterolemic heterozygotes with LDL ≥200 mg/dL AND documented coronary artery disease*

* Documented coronary artery disease: includes a history of myocardial infarction, coronary artery bypass surgery, percutaneous transluminal coronary angioplasty or alternative revascularization procedure, or progressive angina documented by exercise or non-exercise stress test.

LDL apheresis is considered experimental or investigational for all other conditions, including but not limited to treatment of preeclampsia, nonfamilial hypercholesterolemia, sudden sensorineural hearing loss, severe diabetic foot ulcerations, peripheral artery disease, and non‒arteritic acute anterior ischemic optic neuropathy. There is insufficient to clinical evidence to determine the impact of this procedure on health outcomes.

Therapeutic apheresis with selective high-density lipoprotein (HDL) delipidation and plasma reinfusion (0342T) is considered experimental or investigational. The available clinical evidence does not support clinical value.

BILLING/CODING INFORMATION:

CPT Coding:

36511

Therapeutic apheresis; for white blood cells

36512

Therapeutic apheresis; for red blood cells

36513

Therapeutic apheresis; for platelets

36514

Therapeutic apheresis; for plasma pheresis

36515

Therapeutic apheresis; with extracorporeal immunoadsorption and plasma reinfusion

36516

Therapeutic apheresis; with extracorporeal selective adsorption or selective filtration and plasma reinfusion

0342T

Therapeutic apheresis with selective HDL delipidation and plasma reinfusion (investigational)

HCPCS Coding:

S2120

Low density lipoprotein (LDL) apheresis using heparin-induced extracorporeal LDL precipitation

ICD-10 Diagnosis Codes That Support Medical Necessity for 36511-36515:

C88.0

Waldenstrom macroglobulinemia

C88.2

Heavy chain disease

D47.3

Essential (hemorrhagic) thrombocythemia

D58.8

Other specified hereditary hemolytic anemias

D59.3

Hemolytic-uremic syndrome

D69.49

Other primary thrombocytopenia

D69.51

Posttransfusion purpura

D69.59

Other secondary thrombocytopenia

D75.1

Secondary polycythemia

D89.1

Cryoglobulinemia

G35

Multiple sclerosis

G60.9

Hereditary and idiopathic neuropathy, unspecified

G61.0

Guillain-Barre syndrome

G61.81

Chronic inflammatory demyelinating polyneuritis

G70.01

Myasthenia gravis with (acute) exacerbation

M30.1

Polyarteritis with lung involvement [Churg-Strauss]

M31.0

Hypersensitivity angiitis

M31.1

Thrombotic microangiopathy

M31.30

Wegener's granulomatosis without renal involvement

M31.31

Wegener's granulomatosis with renal involvement

O14.10

Severe pre-eclampsia, unspecified trimester

O14.12

Severe pre-eclampsia, second trimester

O14.13

Severe pre-eclampsia, third trimester

O14.20

HELLP syndrome (HELLP), unspecified trimester

O14.22

HELLP syndrome (HELLP), second trimester

O14.23

HELLP syndrome (HELLP), third trimester

O90.89

Other complications of the puerperium, not elsewhere classified

ICD-10 Diagnosis Codes That Support Medical Necessity for 36516, S2120:

E78.01

Familial hypercholesterolemia

REIMBURSEMENT INFORMATION:

Refer to section entitled POSITION STATEMENT.

PROGRAM EXCEPTIONS:

Federal Employee Program (FEP): Follow FEP guidelines.

State Account Organization (SAO): Follow SAO guidelines.

Medicare Advantage products:

The following National Coverage Determination (NCD) was reviewed on the last guideline reviewed date: APHERESIS (Therapeutic Pheresis) (110.14), located at cms.gov.

The following Local Coverage Determinations (LCD) were reviewed on the last guideline reviewed date: Low Density Lipoprotein (LDL) Apheresis (L33381), and Non-Covered Services (L33777), located at fcso.com.

DEFINITIONS:

Familial hypercholesterolemia (FH): A recessive genetic disorder caused by a defect on chromosome 19 that makes the liver incapable of metabolizing (or removing) excess LDL. The result is very high LDL levels which can lead to premature cardiovascular disease. There are two forms of FH. If inherited from one parent, the result is heterozygous FH (HeFH). HeFH occurs in 1 in 200 to 500 people worldwide. If inherited from both parents, it is much more severe in its consequences. This form of FH is called homozygous FH (HoFH). It is very rare, occurring in about 1 in 160,000 to one million people worldwide.

Guillain-Barre syndrome: an acute demyelinating neuropathy whose severity is graded on a scale of 1-5.  Plasma exchange is reserved for those with grades 3-5 disease who do not initially respond to prednisone.

HELLP syndrome of pregnancy: a severe form of preeclampsia, characterized by hemolysis [H], elevated liver enzymes [EL], and low platelet [LP] counts).

Myasthenia gravis: an autoimmune disease with autoantibodies directed against the postsynaptic membrane of the muscle end plate.  Clinically, the disease is characterized by fatigable weakness of voluntary muscles.  Initial treatment focuses on the use of cholinesterase inhibitors to overcome the postsynaptic blockade.  Immunosuppressant drugs including corticosteroids and azathioprine are also effective.  Plasma exchange has been used as a short-term therapy in individuals with acute exacerbations associated with severe weakness.

Post-transfusion purpura: a rare disorder characterized by an acute severe thrombocytopenia occurring about 1 week after a blood transfusion in association with a high titer of anti-platelet alloantibodies.

Rapidly progressive glomerulonephritis (RPGN) including Goodpasture’s syndrome: a general term describing the rapid loss of renal function in conjunction with the finding of glomerular crescents on renal biopsy specimens.  There are multiple etiologies of RPGN including vasculitis, the deposition of anti-glomerular basement membrane (GBM) antibodies as seen in Goodpasture’s syndrome, or the deposition of immune complexes as seen in various infectious diseases or connective tissue diseases.  RPGN may also be idiopathic.  Because many cases of RPGN represent an immune-mediated disease of acute onset, RPGN was an early focus of PE research.

Thrombotic thrombocytopenic purpura (TTP)—Hemolytic uremic syndrome (HUS): Once considered distinct syndromes, TTP and HUS are now considered different manifestations of the same disease process, i.e., thrombotic microangiopathy.  The classic signs and symptoms include fever, thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, and renal involvement. TTP-HUS may be seen in association with other conditions, such as pregnancy or HIV infection. PE has become the primary treatment for TTP-HUS, although a rationale for its effectiveness is unknown. PE is performed daily until a response is noted; the length of treatment averages about once a month, with increasing intervals between PE treatments.

RELATED GUIDELINES:

Extracorporeal Photopheresis, 01-90919-02

OTHER:

None applicable

REFERENCES:

  1. AHRQ National Guideline Clearinghouse. Guideline Summary NGC-8493: British Association of Dermatologists' guidelines for the management of bullous pemphigoid 2012. Br J Dermatol. 2012 Dec;167(6):1200-14.
  2. AHRQ National Guideline Clearinghouse. Guideline Summary NGC-8948. Lipids and lipoproteins. In: Expert panel on integrated guidelines for cardiovascular health and risk reduction in children and adolescents. National Heart, Lung, and Blood Institute; 2011. p. 184-281.
  3. AHRQ National Guideline Clearinghouse. Guideline Summary NGC 10426: Guidelines on the diagnosis and management of Waldenström macroglobulinaemia. 2006 Mar (revised 2014 May). British Committee for Standards in Haematology - Professional Association.
  4. Barth D, Nouri MN, Ng E, Nwe P, Bril V. (2011). Comparison of IVIg and PLEX in patients with myasthenia gravis. Neurology, 76(23), 2017-2023.
  5. Blue Cross Blue Shield Association Medical Policy Reference Manual. 8.02.02, Plasma Exchange (May 2015).
  6. Blue Cross Blue Shield Association Medical Policy Reference Manual. 8.02.04, Lipid Apheresis (August 2015).
  7. Centers for Medicare and Medicaid Services (CMS). Publication 100-3, Manual Section 110.14. APHERESIS (Therapeutic Pheresis). 07/30/92.
  8. ClinicalTrials.gov. NCT01518205: HELP-Apheresis in Diabetic Ischemic Foot Treatment (H.A.D.I.F) (HADIF). December 2014.
  9. ClinicalTrials.gov. NCT01753232: Safety and Efficacy of the DALI LDL-adsorber and MONET Lipoprotein Filter (LINET) (December 2013).
  10. ClinicalTrials.gov. NCT01840683: HELP Therapy for Dry AMD (HELPuc) (November 2014).
  11. Colvin MM, et al. Antibody-mediated rejection in cardiac transplantation: emerging knowledge in diagnosis and management: a scientific statement from the American Heart Association. Circulation. 2015 May 5;131(18):1608-39.
  12. Connelly-Smith LS, Linenberger ML. Therapeutic apheresis for patients with cancer. Cancer Control. 2015 Jan;22(1):60-78.
  13. Cordoba JP, et al. Therapeutic plasma exchange in rheumatic diseases: a university hospital experience. Rev Bras Reumatol. 2016 Dec 16.
  14. Cortese I, Chaudhry V, So YT et al. Evidence-based guideline update: Plasmapheresis in neurologic disorders. Neurology 2011; 76(3):294-300.
  15. Cortese I, Cornblath DR. Therapeutic plasma exchange in neurology: 2012. J Clin Apher. 2013 Feb;28(1):16-9.
  16. Dhaun N, et al. Benefits of an expanded use of plasma exchange for anti-neutrophil cytoplasmic antibody-associated vasculitis within a dedicated clinical service. BMC Musculoskelet Disord. 2015 Nov 9;16:343.
  17. Dorland’s Illustrated Medical Dictionary, 27th Edition.
  18. ECRI, Windows on Technology – “Plasmapheresis for the Treatment of Multiple Sclerosis” (10/01).
  19. El-Bayoumi MA, El-Refaey AM, Abdelkader AM, El-Assmy MM, Alwakeel AA, El-Tahan HM. (2011). Comparison of intravenous immunoglobulin and plasma exchange in treatment of mechanically ventilated children with Guillain Barre syndrome: a randomized study. Crit Care, 15(4), R164.
  20. First Coast Service Options, Inc. Florida Medicare Part B Local Coverage Determination L32998, Low Density Lipoprotein (LDL) Apheresis (Retired 09/30/15).
  21. First Coast Service Options, Inc. Florida Medicare Part B Local Coverage Determination L33381, Low Density Lipoprotein (LDL) Apheresis (10/01/15).
  22. First Coast Service Options, Inc. Florida Medicare Part B Local Coverage Determination L29288, Noncovered Services (02/02/09). (Retired 09/30/15).
  23. First Coast Service Options, Inc. Florida Medicare Part B Local Coverage Determination L33777, Noncovered Services (10/01/15).
  24. Goldberg AC, Hopkins PN, Toth PP, et al. Familial hypercholesterolemia: screening, diagnosis and management of pediatric and adult patients: clinical guidance from the National Lipid Association Expert Panel on Familial Hypercholesterolemia. J Clin Lipidol. Jun 2011;5(3 Suppl):S1-8.
  25. HAYES Medical Technology Directory; “Extracorporeal Immunoadsorption Using Protein A Columns (Prosorba®) for the Treatment of Rheumatoid Arthritis” EXTR0201.18 (06/00).
  26. HAYES Update: “Extracorporeal Immunoadsorption Using Protein A Columns (Prosorba) for the Treatment of Rheumatoid Arthritis”, EXTR0201.18 (11/21/02).
  27. Health Quality Ontario. Low-density lipoprotein apheresis: an evidence-based analysis. Ontario health technology assessment series 7.5 (2007): 1.
  28. Ito MK, Watts GF. Challenges in the Diagnosis and Treatment of Homozygous Familial Hypercholesterolemia. Drugs. 2015 Oct;75(15):1715-24.
  29. Julius U. Lipoprotein apheresis in the management of severe hypercholesterolemia and of elevation of lipoprotein(a): current perspectives and patient selection. Med Devices (Auckl). 2016 Oct 13;9:349-360.
  30. Kavey REW, et al. Cardiovascular Risk Reduction in High-Risk Pediatric Patients. Circulation 114.24 (2006): 2710-2738.
  31. Korsak J, Wańkowicz Z. New Options of Apheresis in Renal Diseases: How and When? Blood Purif. 2016;41(1-3):1-10.
  32. Leebmann J, Roeseler E, Julius U, et al. Lipoprotein apheresis in patients with maximally tolerated lipid-lowering therapy, lipoprotein(a)-hyperlipoproteinemia, and progressive cardiovascular disease: prospective observational multicenter study. Circulation. Dec 17 2013;128(24):2567-2576.
  33. Medicare Coverage Issues Manual (CIM 35-60) (05/97).
  34. National Comprehensive Cancer Network (NCCN). NCCN Guidelines: Multiple Myeloma, Version 2.2015. 09/30/2014. Accessed at http://www.nccn.org/professionals/physician_gls/pdf/myeloma.pdf.
  35. National Institute for Health and Clinical Excellence (NICE). National Collaborating Centre for Primary Care. Identification and management of familial hypercholesterolaemia. London (UK). 2008 Aug. (Clinical guideline; no.71).
  36. O'Donghaile D, Kelley W, Klein HG, Flegel WA. Recommendations for transfusion in ABO-incompatible hematopoietic stem cell transplantation. Transfusion. 2012 Feb;52(2):456-8.
  37. Pagano MB, Murinson BB, Tobian AA, King KE. Efficacy of therapeutic plasma exchange for treatment of stiff-person syndrome. Transfusion, 2014.
  38. Safarova MS, Kullo IJ. My Approach to the Patient With Familial Hypercholesterolemia. Mayo Clin Proc. 2016 Jun;91(6):770-86.
  39. Schettler V, Neumann CL, Hulpke-Wette M, Hagenah GC, Schulz EG, Wieland E; German Apheresis Working Group. Current view: indications for extracorporeal lipid apheresis treatment. Clin Res Cardiol Suppl. 2012 Jun;7(Suppl 1):15-9.
  40. Schmidt JJ, et al. Effect of therapeutic plasma exchange on plasma levels and total removal of adipokines and inflammatory markers. BMC Obes. 2015 Sep 30;2:37.
  41. Schwartz J, Winters JL, Padmanabhan A et al. Guidelines on the use of therapeutic apheresis in clinical practice-evidence-based approach from the Writing Committee of the American Society for Apheresis: the sixth special issue. J Clin Apher 2013; 28(3):145-284.
  42. Schettler VJ, et al; Scientific Board of GLAR for the German Apheresis Working Group. The German Lipoprotein Apheresis Registry (GLAR) - almost 5 years on. Clin Res Cardiol Suppl. 2017 Mar;12(Suppl 1):44-49.
  43. Vlachopanos G, Georgalis A, Gakiopoulou H. Plasma Exchange for the Recurrence of Primary Focal Segmental Glomerulosclerosis in Adult Renal Transplant Recipients: A Meta-Analysis. J Transplant. 2015;2015:639628.
  44. Waksman R, Torguson R, Kent KM, et al. A first-in-man, randomized, placebo-controlled study to evaluate the safety and feasibility of autologous delipidated high-density lipoprotein plasma infusions patients with acute coronary syndrome. J Am Coll Cardiol. Jun 15 2010;55(24):2727-2735.
  45. Walsh M, et al. Plasma exchange for renal vasculitis and idiopathic rapidly progressive glomerulonephritis: a meta-analysis. American journal of kidney diseases: the official journal of the National Kidney Foundation 57.4 (2011): 566.
  46. Wang A, et al. Systematic Review of Low-Density Lipoprotein Cholesterol Apheresis for the Treatment of Familial Hypercholesterolemia. J Am Heart Assoc. 2016 Jul 6;5(7). pii: e003294.
  47. Youngblom E, Pariani M, Knowles JW. Familial Hypercholesterolemia. 2014 Jan 2 [Updated 2016 Dec 8]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2017. Available from: https://www.ncbi.nlm.nih.gov/books/NBK174884/.

COMMITTEE APPROVAL:

This Medical Coverage Guideline (MCG) was approved by the BCBSF Medical Policy & Coverage Committee on 06/22/17.

GUIDELINE UPDATE INFORMATION:

11/15/00

Medical Coverage Guideline developed.

04/15/01

Revised to include coverage information for ECI.

08/28/02

Revised to include ICD-9 diagnosis code for a covered indication for 36521; Add clarification for ICD-9 diagnosis codes that support medical necessity for 36520.

01/01/03

HCPCS coding update.

04/15/03

Reviewed; revised to include additional covered indication.

05/15/07

Medical Coverage Guideline archived.

02/15/15

Medical Coverage Guideline returned to active status. Revised MCG title, description section, position statement, CPT, HCPCS, ICD9, and ICD10 coding; Medicare Advantage program exception, and definitions. Updated references and reformatted guideline.

10/01/15

Revision; updated ICD9 coding section.

02/15/16

Scheduled review. Maintained position statement, revised program exceptions section, and updated references.

10/01/16

ICD-10 coding update: deleted code E78.0; added code E78.01.

11/17/16

Unscheduled revision. Deleted ICD10 code G70.00.

03/15/17

Scheduled review. Maintained position statement. Updated references. Reformatted guideline.

07/15/17

Revision: Revised description section, coverage for low-density lipoprotein (LDL) apheresis, and definitions section. Updated references.

Date Printed: December 18, 2017: 11:42 AM