The proposal is to add words in red text and to delete words or statements with a strikethrough:

Transcription

1 Plicy Medical Plicy Manual Draft Revised Plicy: D Nt Implement Plasma Exchange DESCRIPTION Plasma exchange (PE) is a prcedure in which the bld f the patient is extracted and prcessed thrugh a medical device which separates the plasma frm ther cmpnents f bld. The plasma is discarded and replaced with fluid such as albumin, allgenic plasma r a plasma substitute. PE is a nnspecific therapy, because the entire plasma is discarded. PE has been used in a wide variety f acute and chrnic cnditins, as well as in the setting f slid rgan transplantatin. The terms therapeutic apheresis, plasmapheresis, and plasma exchange are ften used interchangeably, but when prperly used dente different prcedures. The American Sciety fr Apheresis (ASFA) definitins fr these ther prcedures are as fllws: Apheresis: A prcedure in which whle bld f the patient r dnr is passed thrugh a medical device which separates ut any f the particular cmpnents f the bld and returns the remainder t circulatin, with r withut extracrpreal treatment r replacement f the separated cmpnent. Plasmapheresis: A prcedure in which whle bld is passed thrugh a medical device that separates the plasma frm the ther cmpnents f the bld. The red and white bld cells are transfused back int the persn, the plasma is discarded withut a replacement slutin. The prpsed therapeutic use f PE is t rapidly remve txins r autantibdies which can accumulate in the plasma as a result f a disease prcess. PE is a nn-specific therapy and essentially a symptmatic treatment since it des nt remve the surce f the pathgenic factrs. Althugh PE can rapidly reduce levels f serum autantibdies; a feedback mechanism may lead t a rebund verprductin f the same antibdies. Nte: Applicatins f PE can be bradly subdivided int 2 general categries: (1) acute self-limited diseases, in which PE is used t lwer the circulating pathgenic substance; and (2) chrnic diseases, in which there is nging prductin f pathgenic autantibdies. Treatment gals and duratin f treatment with PE need t be clearly established befre its initiatin; withut such treatment gals, the use f an acute shrt-term curse f PE may insidiusly evlve t a chrnic use f PE with uncertain benefit. Fr example a meaningful clinical respnse fr chrnic inflammatry demyelinating plyneurpathy (CIDP) wuld be an imprvement in neurlgical disability scres (e.g. Hughes GBS Disability Scale, Overall Disability Sum Scre). Hwever, studies shw prgress made will mst likely last nly fr the shrt term. Anther example wuld be plasma exchange when used t treat myasthenia gravis r Guillain-Barre wuld shw measurable imprvement in bedside spirmetry readings. Additinally neurmyelitis ptica and neurmyelitis ptica spectrum disrder (NMO/NMOSD), a rare but clinically aggressive demyelinating disease f the central nervus system, that des nt respnd t initial treatment f sterids may respnd t a shrt curse f plasma exchange treatments, especially if previus treatments resulted in imprvements in visual acuity r disability scres. POLICY The prpsal is t add wrds in red text and t delete wrds r statements with a strikethrugh: Plasma exchange is cnsidered medically necessary fr specified diseases/cnditins if the medical apprpriateness criteria are met. (See Medical Apprpriateness belw.) Plasma exchange fr the treatment f all ther diseases/cnditins including, but nt limited t the fllwing is cnsidered investigatinal: This dcument has been classified as public infrmatin

2 Plicy Medical Plicy Manual Draft Revised Plicy: D Nt Implement ABO incmpatible slid rgan transplant; liver Acute disseminated encephalpathy Acute inflammatry demyelinating plyneurpathy (Guillain-Barré syndrme) in children yunger than 10 with mild r mderate frms Acute liver failure Amytrphic lateral sclersis Aplastic anemia Asthma Autimmune hemlytic anemia; warm hemlytic anemia; cld agglutinin disease Bullus pemphigid Cardimypathy, pst-transplant Central pntine myelinlysis Chrnic fatigue syndrme Cagulatin factr inhibitrs Cryglbulinemia; (except fr severe mixed cryglbulinemia, as nted belw) Heart transplant antibdy mediated rejectin Hemlytic uremic syndrme (HUS) with typical presentatin (diarrhea related) Idipathic thrmbcytpenic purpura (ITP); refractry r nn-refractry Inclusin bdy mysitis Lambert-Eatn myasthenic syndrme Mild Guillain-Barré syndrme Multiple sclersis; chrnic prgressive r relapsing remitting Mushrm pisning Myasthenia gravis with anti-musk antibdies Obsessive-cmpulsive disrder and tic disrder f childhd Overdse and pisning (ther than mushrm) Paraneplastic syndrmes Paraprteinemic plyneurpathy, (IgM) Pediatric autimmune neurpsychiatric disrders assciated with streptcccal infectins (PANDAS) Pemphigus vulgaris Phytanic acid strage disease (Refsum s disease) POEMS (plyneurpathy, rganmegaly, endcrinpathy, M prtein, skin changes) Plymysitis and dermatmysitis Psriasis Raynaud's phenmenn Red cell allimmunizatin in pregnancy Reginal enteritis (Crhn's disease) Rheumatid arthritis Sclerderma (systemic sclersis) Severe late left ventricular failure Stiff persn syndrme Sydenham s chrea (SC) Systemic lupus erythematsus; manifestatins ther than nephritis Systemic Lupus nephritis Systemic vasculitis t imprve renal functin Thyrtxicsis MEDICAL APPROPRIATENESS Plasma exchange is cnsidered medically apprpriate if ANY ONE f the fllwing are met: Autimmune This dcument has been classified as public infrmatin

3 Plicy Medical Plicy Manual Draft Revised Plicy: D Nt Implement Severe multiple manifestatin f mixed cryglbulinemia (e.g. cryglbulinemic nephrpathy, skin ulcers, sensry mtr neurpathy and widespread vasculitis) used in cnjunctin with immunsuppressive treatment Catastrphic anti-phsphlipid syndrme (CAPS) Pediatric pst-infectius autimmune neurpsychiatric disrders (e.g., PANDAS, Sydenham s Chrea; which typically fllw Grup-A beta-hemlytic streptcccus infectin) Wilsn s Disease, fulminant (autsmal recessive genetic disrder resulting in cpper accumulatin in the liver, brain, crnea, and kidney) Hematlgic ABO incmpatible hematpietic prgenitr cell transplantatin Hyperviscsity syndrme and ANY ONE f the fllwing: multiple myelma Waldenström's macrglbulinemia Thrmbtic thrmbcytpenia purpura (TTP) Idipathic thrmbcytpenia purpura (ITP) in emergency situatin Atypical hemlytic-uremic syndrme (HUS) Pst transfusin purpura HELLP syndrme f pregnancy (a severe frm f preeclampsia, characterized by hemlysis [H], elevated liver enzymes [EL], and lw platelet [LP] cunts) Myelma with acute renal failure IgA r IgG paraprteinemia plyneurpathy Transplant Slid rgan transplantatin with ABO incmpatibility r psitive crssmatch demnstrated by high panel reactive antibdy (PRA) screen, when a suitable nnreactive live r cadaveric dnr is unavailable in ANY ONE f the fllwing: Kidney Heart (infant) Liver Acute allgraft cardiac rejectin Fcal segmental glmerulsclersis nly when fllwing renal transplant Renal Anti-glmerular basement membrane antibdies (i.e., Gdpasture's syndrme) with prgressive renal failure Antineutrphil cytplasmic antibdy (ANCA) assciated vasculitis (e.g., Wegner s granulmatsis) with assciated renal failure (serum CR abve 5.8 mg/dl) Dense depsit disease with Factr H deficiency and/r elevated C3 Nephritic factr Neurlgic Acute fulminant central nervus system demyelinatin (e.g., Acute Multiple Sclersis, Acute Transverse Myelitis) Severe Guillain-Barré syndrme with ANY ONE f the fllwing: Inability t walk 5 meters withut assistance Cnfinement t bed / chair-bund Ventilatin assistance fr at least part f the day r night This dcument has been classified as public infrmatin

4 Plicy Medical Plicy Manual Draft Revised Plicy: D Nt Implement Acute neurmyelitis ptica (NMO) with ALL f the fllwing met: Failed high-dse intravenus crticsterids Chrnic neurmyelitis ptica (NMO) with ALL f the fllwing met: Failed high-dse crticsterids Failed r nt medically apprpriate azathiprine with r withut lw-dse intravenus crticsterids Failed r nt medically apprpriate mycphenlate mfetil with r withut lw-dse intravenus crticsterids Failed r nt medically apprpriate rituximab Myasthenia gravis in crisis r as part f a preperative preparatin Chrnic inflammatry demyelinating plyneurpathy (CIDP) with ALL f the fllwing: Severe r life-threatening symptms Failed respnse t cnventinal therapy with prednisne r intravenus immunglbulins (IVIg) Meet the cnfirmatry criteria fr CIDP develped by the American Academy f Neurlgy AIDS Task Frce which includes ALL f the fllwing: Nerve bipsy shwing unequivcal evidence f demyelinatin and remyelinatin f prximal nerve segments in which the predminant prcess is demyelinatin Demyelinatin by either electrn micrscpy (greater than 5 fibers) r teased fiber studies (greater than 12% f 50 teased fibers, minimum f fur interndes each, demnstrating demyelinatin/remyelinatin) CSF study - cell cunt less than 10/mm3 if HIV-sernegative r less than 50/mm3 if HIV serpsitive CSF study - negative VDRL Prgressive r relapsing mtr and sensry, rarely n mtr r sensry, dysfunctin f mre than ne limb r a peripheral nerve nature, develping ver at least 2 mnths Nerve cnductin studies including studies f prximal nerve segments in which the predminant prcess is demyelinatin Nerve cnductin studies with 3 r mre f the fllwing: Reductin in cnductin velcity (CV) in tw r mre mtr nerves with ANY ONE f the fllwing: Less than 80% f lwer limit f nrmal (LLN) if amplitude greater than 80% f LLN Less than 70% f LLN if amplitude less than 80% f LLN Partial cnductin blck r abnrmal tempral dispersin in ne r mre mtr nerves: either perneal nerve between ankle and belw fibular head, median nerve between wrist and elbw, r ulnar nerve between wrist and belw elbw with ANY ONE f the fllwing: Criteria suggestive f partial cnductin blck: less than 15% change in duratin between prximal and distal sites and greater than 20% drp in negative peak (-p) area r peak t peak (p-p) amplitude between prximal and distal sites Criteria fr abnrmal tempral dispersin and pssible cnductin blck: greater than 15% change in duratin between prximal and distal sites and greater than 20% drp in -p area r p-p amplitude between prximal and distal sites and greater than 20% drp in -p r p-p amplitude between prximal and distal sites. These criteria are nly suggestive f partial cnductin blck as they are derived frm studies f nrmal individuals. Additinal studies, such as stimulatin acrss shrt segments r recrding f individual mtr unit ptentials, are required fr cnfirmatin. Prlnged distal latencies in tw r mre nerves with ANY ONE f the fllwing: This dcument has been classified as public infrmatin

5 Plicy Medical Plicy Manual Draft Revised Plicy: D Nt Implement Greater than 125% f upper limit f nrmal (ULN) if amplitude greater than 80% f LLN Greater than 150% f ULN if amplitude less than 80% f LLN Absent F-waves r prlnged minimum F-wave latencies (10 t 15 trials) in tw r mre mtr nerves with ANY ONE f the fllwing: Greater than 120% f ULN if amplitude greater than 80% f LLN Greater than 150% f ULN if amplitude less than 80% f LLN IMPORTANT REMINDERS Any specific prducts referenced in this plicy are just examples and are intended fr illustrative purpses nly. It is nt intended t be a recmmendatin f ne prduct ver anther, and is nt intended t represent a cmplete listing f all prducts available. These examples are cntained in the parenthetical e.g. statement. We develp Medical Plicies t prvide guidance t Members and Prviders. This Medical Plicy relates nly t the services r supplies described in it. The existence f a Medical Plicy is nt an authrizatin, certificatin, explanatin f benefits, r a cntract fr the service (r supply) that is referenced in the Medical Plicy. Fr a determinatin f the benefits that a Member is entitled t receive under his r her health plan, the Member's health plan must be reviewed. If there is a cnflict between the Medical Plicy and a health plan, the express terms f the health plan will gvern. ADDITIONAL INFORMATION Based n data frm published studies and/r clinical supprt, plasma exchange is cnsidered medically necessary fr selected cnditins. Fr all ther cnditins, there is a lack f data regarding efficacy and clinical supprt. SOURCES American Academy f Neurlgy. (2016). Cntinuum. Multiple sclersis and ther demyelinating diseases. Retrieved June 30, 2016 frm American Sciety fr Apheresis (2016) Guidelines n the use f therapeutic apheresis in clinical practice evidence-based apprach frm the writing cmmittee f the american sciety fr apheresis: the seventh special issue. Jurnal f Clinical Apheresis, 31, Bambauer, R., Latza, R., Bambauer, C., Burgard, D., and Schiel, R. (2013, Nvember) Therapeutic apheresis in autimmune diseases. Rheumatlgy: Research and Reviews, 5, (Level 2 evidence) Bnnan, M., & Cabre, P. (2012). Plasma exchange in severe attacks f neurmyelitis ptica. Multiple Sclersis Internatinal, 2012, Article ID (Level 1 evidence) Centers fr Medicare & Medicaid Services. CMS.gv. NCD fr apheresis (110.14). Retrieved January 8, 2016 frm Cdrn, P., Cusin, M., Subra, J., Pautt, V., Leturnel, F., Verny, C., et. al., (2017, March) Therapeutic plasma exchange in chrnic dysimmune peripheral neurpathies: A 10-year retrspective study. Jurnal f Clinical Apheresis, [e-published ahead f print] Abstract retrieved July 18, 2017 frm PubMed database. Deschamps, R., Gueguen, A., Parquet, N., Saheb, S., Driss, F., Mesnil, M., et. al. (2016, May) Plasma exchange respnse in 34 patients with severe ptic neuritis. Jurnal f Neurlgy, 263 (5) Abstract retrieved July 18, 2017 frm PubMed database. This dcument has been classified as public infrmatin

6 Plicy Medical Plicy Manual Draft Revised Plicy: D Nt Implement Grsn, L. (2012) An update n the management f chrnic inflammatry demyelinating plyneurpathy. Therapeutic Advances in Neurlgical Disrders, 5 (6), (Level 5 evidence) Kbayashim, M., Nanri, K., Taguchi, T., Ishik, T., Yshida, M., Yshikawa, N. et. al., (2015, February) Immunadsrptin therapy fr neurmyelitis ptica spectrum disrders lng after the acute phase. Jurnal f Apheresis, 30 (1), Abstract retrieved July 18, 2017 frm PubMed database. Mehndiratta, M,, Hughes, R., and Pritchard. J. (2015) Plasma exchange fr chrnic inflammatry demyelinating plyradiculneurpathy. Cchrane Database f Systematic Reviews, Issue 8. Art. N.: CD (Level 2 evidence) Natinal Cmprehensive Cancer Netwrk. (2017, January). NCCN clinical practice guidelines in nclgy. Waldenstrm s macrglbulinemia/lymphplasmacytic lymphma - V Retrieved April 27, 2017 frm Natinal Cmprehensive Cancer Netwrk. (2018, February). NCCN clinical practice guidelines in nclgy (NCCN Guidelines ). Multiple myelma - V Retrieved July 20, 2018 frm Nickersn, M and Marrie, R. (2013) The multiple sclersis relapse experience: patient-reprted utcmes frm the Nrth American Research Cmmittee n Multiple Sclersis (NARCOMS) Registry. BMC Neurlgy, 13 (119). (Level 4 evidence) Oaklander, A., Lunn, M., Hughes, R., van Schaik, I., Frst, C., Chalk, C., et. al (2017, January) Treatments fr chrnic inflammatry demyelinating plyradiculneurpathy (CIDP): an verview f systematic reviews. Cchrane Database Systems Review. January, 13; 1:CD Abstract retrieved April 27, 2017 frm PubMed database. Sherman, E. and Han, M. (2015) Acute and chrnic management f neurmyelitis ptica spectrum disrder. Current Treatment Optins in Neurlgy, 17 (48). (Level 2 evidence) U. S. Fd and Drug Administratin. (2004, July). Center fr Devices and Radilgical Health. 510(k) Pre-market Ntificatin Database. K (Apheresis System). Retrieved Nvember 15, 2010 frm Walsh, M., Catapan, F., Szpirt, W., Thrlund, K., Bruchfeld, A., Guillevin, L. (2011). Plasma exchange fr renal vasculitis and idipathic rapidly prgressive glmerulnephritis: a meta-analysis. American Jurnal f Kidney Disease, 57 (4), (Level 1 evidence - Independent) EFFECTIVE DATE ID_BT This dcument has been classified as public infrmatin