Protocol. Plasma Exchange

Transcription

1 Protocol Plasma Exchange (80202) Medical Benefit Effective Date: 01/01/16 Next Review Date: 09/18 Preauthorization Yes Review Dates: 03/07, 05/08, 05/09, 05/10, 05/11, 05/12, 07/12, 05/13, 05/14, 09/14, 09/15, 09/16, 09/17 Preauthorization is required. The following protocol contains medical necessity criteria that apply for this service. The criteria are also applicable to services provided in the local Medicare Advantage operating area for those members, unless separate Medicare Advantage criteria are indicated. If the criteria are not met, reimbursement will be denied and the patient cannot be billed. Please note that payment for covered services is subject to eligibility and the limitations noted in the patient s contract at the time the services are rendered. Description Plasma exchange (PE) is a procedure in which the plasma is isolated, then discarded and replaced with a substitution fluid such as albumin. PE 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. Summary of Evidence Due to data from published studies and/or clinical support, plasma exchange is considered medically necessary for selected conditions. For conditions in which there is a lack of efficacy data and clinical support, plasma exchange is considered investigational. Policy Plasma exchange (PE) is considered medically necessary for the conditions listed next. 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. 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; Page 1 of 9

2 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. Neurologic acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome [GBS]; severity grade 1 2 within two weeks of onset; severity grade 3 5 within four 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 anti-glomerular basement membrane disease (Goodpasture s syndrome); ANCA [antineutrophil cytoplasmic antibody]-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; o o Kidney; Heart (infants); renal transplantation: antibody mediated rejection; HLA [human leukocyte antigen] desensitization; focal segmental glomerulosclerosis after renal transplant. PE 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-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 s 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; Page 2 of 9

3 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 non-refractory; inclusion body myositis; Lambert-Eaton myasthenic syndrome; multiple sclerosis with chronic progressive or relapsing remitting course; neuromyelitis optica (NMO); 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 s chorea (SC); systemic lupus erythematosus (including SLE [systemic lupus erythematous] nephritis); thyrotoxicosis; and hyperviscosity syndromes with renal failure (other than associated with multiple myeloma or Waldenstrom s macroglobulinemia). Policy Guidelines Patients receiving PE as a treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) should meet the diagnostic criteria for CIDP. Criteria were published in the Task Force Report of the Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force (Neurology 1991; 41:617-18). The Page 3 of 9

4 diagnostic criteria have not been officially updated since The use of PE in patients with acute, life-threatening complications of chronic autoimmune diseases, such as rheumatoid arthritis and SLE, may need to be considered on an individual basis. An example of such a situation would be the development of a severe vasculitis, in which it is hoped that the use of PE can acutely lower the level of serum autoantibodies until an alternate long-term treatment strategy can be implemented. However, in these situations, the treatment goals and duration of treatment with PE need to be clearly established before its initiation; without such treatment goals, the use of an acute short-term course of PE may insidiously evolve to a chronic use of PE with uncertain benefit. Medicare Advantage Please see Medicare Advantage Policy Guidelines for additional pertinent information. For Medicare Advantage apheresis is medically necessary for the following indications: Plasma exchange for acquired myasthenia gravis; Plasmapheresis in the treatment of primary macroglobulinemia (Waldenström); Treatment of hyperglobulinemias, including (but not limited to) multiple myelomas, cryoglobulinemia and hyperviscosity syndromes; Plasmapheresis or plasma exchange as a last resort treatment of thrombotic thrombocytopenic purpura (TTP); Plasmapheresis or plasma exchange in the last resort treatment of life threatening rheumatoid vasculitis; Plasma perfusion of charcoal filters for treatment of pruritus of cholestatic liver disease; Plasma exchange in the treatment of Goodpasture s Syndrome; Plasma exchange in the treatment of glomerulonephritis associated with antiglomerular basement membrane antibodies and advancing renal failure or pulmonary hemorrhage; Treatment of chronic relapsing polyneuropathy for patients with severe or life threatening symptoms who have failed to respond to conventional therapy; Treatment of life threatening scleroderma and polymyositis when the patient is unresponsive to conventional therapy; Treatment of Guillain-Barré Syndrome; Treatment of last resort for life threatening systemic lupus erythematosus (SLE) when conventional therapy has failed to prevent clinical deterioration. Medicare Advantage Policy Guidelines Settings When Apheresis meets medical criteria, it is covered only when performed in a hospital setting (either inpatient or outpatient) or in a non-hospital setting, e.g., a physician directed clinic when all of the following conditions are met: A physician (or a number of physicians) is present to perform medical services and to respond to medical emergencies at all times during patient care hours; Page 4 of 9

5 Each patient is under the care of a physician; and All non-physician services are furnished under the direct, personal supervision of a physician. Background The terms therapeutic apheresis, plasmapheresis, and PE are often used interchangeably, but when properly used denote different procedures. The American Society for Apheresis 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., < 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 protocol addresses only PE as a therapeutic apheresis procedure. The rationale for PE 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 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 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 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). Regulatory Status FDA 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. Page 5 of 9

6 The compliance program covers products intended for use both in injectable drug products (e.g., immune globulin, albumin) and noninjectable products (e.g., in vitro devices such as blood bank reagents). Compliance Program Guidance Manual Chapter 42-Blood and Blood Products Product Code for Therapeutic exchange plasma (TEP): 57DI-65 Related Protocol Lipid Apheresis Services that are the subject of a clinical trial do not meet our Technology Assessment Protocol criteria and are considered investigational. For explanation of experimental and investigational, please refer to the Technology Assessment Protocol. It is expected that only appropriate and medically necessary services will be rendered. We reserve the right to conduct prepayment and postpayment reviews to assess the medical appropriateness of the above-referenced procedures. Some of this protocol may not pertain to the patients you provide care to, as it may relate to products that are not available in your geographic area. References We are not responsible for the continuing viability of web site addresses that may be listed in any references below. 1. US Food and Drug Administration (FDA). Compliance Program Guidance Manual; Chapter 42 - Blood and Blood Products 2011; ies/enforcement/complianceprograms/ucm htm. Accessed March, Shumak KH, Rock GA. Therapeutic plasma exchange. N Engl J Med. 1984; 310(12): Lewis EJ, Hunsicker LG, Lan SPActoptisln, et al. The Lupus Nephritis Collaborative Study Group. N Engl J Med. 1992; 326(21): Danieli MG, Palmieri C, Salvi A, et al. Synchronised therapy and high-dose cyclophosphamide in proliferative lupus nephritis. J Clin Apheresis. 2002; 17(2): Khatri BO, McQuillen MP, Harrington GJ, et al. Chronic progressive multiple sclerosis: double-blind controled study of plasmapheresis in patients taking immunosuppressive drugs. Neurology. 1985; 35(3): Weiner HL, Dau PC, Khatri BOD-bsotv, et al. Sham plasma exchange in patients treated with immunosuppression for acute attacks of multiple sclerosis. Neurology. 1989; 39(9): The Canadian Cooperative Multiple Sclerosis Study Group. The Canadian cooperative trial of cyclophosphamide and plasma exchange in progressive multiple sclerosis. Lancet. 1991; 337(8739): Tim RW, Massey JM, Sanders DB. Lambert-Eaton myasthenic syndrome: electrodiagnostic findings and response to treatment. Neurology. 2000; 54(11): Sanders DB, Massey JM, Sanders LL, et al. A randomized trial of 3, 4-diaminopyridine in Lambert-Eaton myasthenic syndrome. Neurology. 2000; 54(3-Jan): Anderson NE, Rosenblum MK, Posner JB. Paraneoplastic cerebellar degeneration: clinical-immunological correlations. Ann Neurol. 1988; 24(4): Dwosh IL, Giles AR, Ford PMPtira, et al. A controlled, double-blind, crossover trial. N Engl J Med. 1983; 308(19): Page 6 of 9

7 12. Miller FW, Leitman SF, Cronin ME, et al. Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med. 1992; 326(21): Guillaume JC, Roujeau JC, Morel P, et al. Controlled study of plasma exchange in pemphigus. Arch Dermatol. Nov 1988; 124(11): PMID Vicari AM, Folli F, Pozza G, et al. Plasmapheresis in the treatment of stiff-man syndrome. N Engl J Med. 1989; 320(22): Brashear HR, Phillips LH. Autoantibodies to GABAergic neurons and response to plasmapheresis in stiff-man syndrome. Neurology. 1991; 41(10): Harding AE, Thompson PD, Kocen RS, et al. Plasma exchange and immunosuppression in the stiff man syndrome. Lancet. 1989; 2(8668): Pagano MB, Murinson BB, Tobian AA, et al. Efficacy of therapeutic plasma exchange for treatment of stiffperson syndrome. Transfusion. Jul 2014; 54(7): PMID Rockx MA, Clark WF. Plasma exchange for treating cryoglobulinemia: a descriptive analysis. Transfus Apher Sci. 2010; 42(3): Michael M, Elliott EJ, Craig JC, et al. Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials. Am J Kidney Dis. 2009; 53(2): Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med. 2009; 361(17): PMID 21. Raphael JC, Chevret S, Hughes RA, et al. Plasma exchange for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2012; 7:CD PMID Hughes RA, Swan AV, Raphaël JC, et al. Immunotherapy for Guillain-Barré syndrome: a systematic review. Brain. 2007; 130(pt 9): Hughes RA, Wijdicks EF, Barohn RQSSotAAoN, et al. Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003; 61(6-Jan): El-Bayoumi MA, El-Refaey AM, Abdelkader AM, et al. Comparison of intravenous immunoglobulin and plasma exchange in treatment of mechanically ventilated children with Guillain Barre syndrome: a randomized study. Crit Care. 2011; 15(4):R Mehndiratta MM, Hughes RA. Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2012; 9:CD PMID Weinshenker BG, O Brien PC, Petterson TM, et al. A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol. 1999; 46(6): Dyck PJ, Low PA, Windebank AJ, et al. Plasma exchange in polyneuropathy associated with monoclonal gammopathy of undetermined significance. N Engl J Med. 1991; 325(21): Kohler W, Bucka C, Klingel R, et al. A randomized and controlled study comparing immunoadsorption and plasma exchange in myasthenic crisis. J Clin Apher. 2011; 26(6): Liu J, Wang W, Zhao C, et al. Comparing the autoantibody levels and clinical efficacy of double filtration plasmapheresis, immunoadsorption and intravenous immunoglobulin for the treatment of late-onset myasthenia gravis. Ther Apher Dial. 2010; 14(2): Barth D, Nabavi Nouri M, Ng E, et al. Comparison of IVIg and PLEX in patients with myasthenia gravis. Neurology. Jun ; 76(23): PMID Ebadi H, Barth D, Bril V. Safety of plasma exchange therapy in patients with myasthenia gravis. Muscle Nerve. Apr 2013; 47(4): PMID Abboud H, Petrak A, Mealy M, et al. Treatment of acute relapses in neuromyelitis optica: Steroids alone versus steroids plus plasma exchange. Mult Scler. Apr PMID Bonnan M, Valentino R, Olindo S, et al. Plasma exchange in severe spinal attacks associated with neuromyelitis optica spectrum disorder. Mult Scler. Apr 2009; 15(4): PMID Merle H, Olindo S, Jeannin S, et al. Treatment of optic neuritis by plasma exchange (add-on) in neuromyelitis optica. Arch Ophthalmol. Jul 2012; 130(7): PMID Page 7 of 9

8 35. National Institute for Neurological Disorders and Stroke (NINDS). NINDS Neuromyelitis Optica Information Page. Accessed March 30, Couser WG. Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy. Am J Kidney Dis. 1988; 11(6): Cole E, Cattran D, Magil AAprtopeaatiicg, et al. The Canadian Apheresis Study Group. Am J Kidney Dis. 1992; 20(3): Walsh M, Catapano F, Szpirt W, et al. Plasma exchange for renal vasculitis and idiopathic rapidly progressive glomerulonephritis: a meta-analysis. Am J Kidney Dis. 2011; 57(4): Jayne DR, Gaskin G, Rasmussen N, et al.; European Vasculitis Study Group. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol. 2007; 18(7): Walsh M, Casian A, Flossmann O, et al. Long-term follow-up of patients with severe ANCA-associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear. Kidney Int. Aug 2013; 84(2): PMID Montgomery RA, Zachary AA. Transplanting patients with a positive donor-specific crossmatch: a single center s perspective. Pediatr Transpl. 2004; 8(6): Jordan SC, Vo AA, Tyan D, et al. Current approaches to treatment of antibody-mediated rejection. Pediatr Transplant. Jun 2005; 9(3): PMID Lehrich RW, Rocha PN, Reinsmoen N, et al. Intravenous immunoglobulin and plasmapheresis in acute humoral rejection: experience in renal allograft transplantation. Hum Immunol. 2005; 66(4): Ibernon M, Gil-Vernet S, Carrera M, et al. Therapy with plasmapheresis and intravenous immunoglobulin for acute humoral rejection in kidney transplantation. Transplant Proc. 2005; 37(9): Gubensek J, Buturovic-Ponikvar J, Kandus A, et al. Plasma exchange and intravenous immunoglobulin in the treatment of antibody-mediated rejection after kidney transplantation: a single-center historic cohort study. Transplant Proc. May 2013; 45(4): PMID Ellingsen I, Florvaag E, Andreassen AH, et al. Plasmapheresis in the treatment of steroid-dependent bronchial asthma. Allergy. 2001; 56(12): Rimmer E, Houston BL, Kumar A, et al. The efficacy and safety of plasma exchange in patients with sepsis and septic shock: a systematic review and meta-analysis. Crit Care. Dec ; 18(6):699. PMID Perlmutter SJ, Leitman SF, Garvey MA, et al. Therapeutic plasma exchange and intravenous immunoglobulin for obsessive-compulsive disorder and tic disorders in childhood. Lancet. 1999; 354(9185): Garvey MA, Snider LA, Leitman SF, et al. Treatment of Sydenham s chorea with intravenous immunoglobulin, plasma exchange, or prednisone. J Child Neurol. 2005; 20(5): National Comprehensive Cancer Network (NCCN). NCC Guidelines Multiple Myeloma, Version Accessed March, Cortese I, Chaudhry V, So YT, et al. Evidence-based guideline update: Plasmapheresis in neurologic disorders. Neurology. 2011; 76(3): 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. Jul 2013; 28(3): PMID Szczepiorkowski ZM, Winters JL, Bandarenko N, et al. Guidelines on the use of therapeutic apheresis in clinical practice--evidence-based approach from the Apheresis Applications Committee of the American Society for Apheresis. J Clin Apher. 2010; 25(3): PMID Page 8 of 9

9 54. Centers for Medicare and Medicaid Services (CMS). National Coverage determination (NCD) for apheresis (therapeutic pheresis) (110.14). e&keywordsearchtype=and&ncd_id=110.14&ncd_version=1&basket=ncd%25253a110%25252e14%25253 A1%25253AApheresis+%252528Therapeutic+Pheresis%252529&bc=gAAAABAAAAAAAA%3d%3d&. Accessed March, Page 9 of 9