American Society for Apheresis 2019 indications for therapeutic apheresis and cytapheresis procedures

Indication	Modality	Category	Evidence
Acute disseminated encephalomyelitis (ADEM): Steroid refractory	TPE	II	2C
Acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barré syndrome): Primary treatment	TPE	I	1A
	IA	I	1B
Acute liver failure	TPE	III	2B
Acute liver failure	TPE-HV	I	1A
Age-related macular degeneration, dry: High-risk	Rheopheresis	II	2B
Amyloidosis, systemic
Dialysis-related amyloidosis	Beta2-microglobulin column	II	2B
Other causes	TPE	IV	2C
Anti-glomerular basement membrane disease (Goodpasture syndrome)
Diffuse alveolar hemorrhage (DAH)	TPE	I	1C
Dialysis-independence	TPE	I	1B
Dialysis-dependence, no DAH	TPE	III	2B
Atopic (neuro-) dermatitis (atopic eczema), recalcitrant	ECP	III	2A
Atopic (neuro-) dermatitis (atopic eczema), recalcitrant	IA/TPE/DFPP	III	2C
Autoimmune hemolytic anemia (AIHA)
Severe cold agglutinin disease	TPE	II	2C
Severe warm AIHA	TPE	III	2C
Babesiosis: Severe	RBC exchange	II	2C
Burn shock resuscitation	TPE	III	2B
Cardiac neonatal lupus	TPE	III	2C
Catastrophic antiphospholipid syndrome (CAPS)	TPE	I	2C
Chronic focal encephalitis (Rasmussen encephalitis)	TPE	III	2C
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)	TPE/IA	I	1B
Coagulation factor inhibitors	TPE	III	2C
Coagulation factor inhibitors	IA	III	2B
Complex regional pain syndrome: Chronic	TPE	III	2C
Cryoglobulinemia: Severe/symptomatic	TPE	II	2A
Cryoglobulinemia: Severe/symptomatic	IA	II	2B
Cutaneous T cell lymphoma (CTCL); mycosis fungoides; Sézary syndrome
Erythrodermic	ECP	I	1B
Non-erythrodermic	ECP	III	2C
Dilated cardiomyopathy, idiopathic: NYHA II-IV	IA	II	1B
Dilated cardiomyopathy, idiopathic: NYHA II-IV	TPE	III	2C
Erythropoietic protoporphyria, liver disease	TPE/RBC exchange	III	2C
Familial hypercholesterolemia
Homozygotes	LA	I	1A
Heterozygotes	LA	II	1A
Homozygotes/heterozygotes	TPE	II	1B
Focal segmental glomerulosclerosis (FSGS)
Recurrent in kidney transplant	TPE/IA	I	1B
Recurrent in kidney transplant/steroid resistant in native kidney	LA	II	2C
Steroid resistant in native kidney	TPE	III	2C
Graft-versus-host disease (GVHD)
Acute	ECP	II	1C
Chronic	ECP	II	1B
Hemolysis, elevated liver enzymes, and low platelets syndrome (HELLP syndrome)
Antepartum	TPE	IV	2C
Postpartum	TPE	III	2C
Hemophagocytic lymphocytosis (HLH); hemophagocytic syndrome; macrophage activating syndrome	TPE	III	2C
Heparin-induced thrombocytopenia and thrombosis (HIT/HITT): Pre-cardiopulmonary bypass or with thrombosis	TPE	III	2C
Hereditary hemochromatosis	Erythrocytapheresis	I	1B
Hyperleukocytosis
Prophylactic or secondary	Leukocytapheresis	III	2C
Symptomatic	Leukocytapheresis	II	2B
Hypertriglyceridemic pancreatitis
Severe	TPE/LA	III	1C
Prevention of relapse	TPE/LA	III	2C
Hyperviscosity in hypergammaglobulinemia
Symptomatic	TPE	I	1B
Prophylaxis for rituximab	TPE	I	1C
IgA nephropathy (Berger's disease)
Chronic progressive	TPE	III	2C
Crescentic	TPE	III	2B
Immune thrombocytopenia (ITP): Refractory	TPE/IA	III	2C
Inflammatory bowel disease
Crohn disease	ECP	III	2C
Ulcerative colitis/Crohn disease	Adsorptive cytapheresis	III	1B
Lambert-Eaton myasthenic syndrome	TPE	II	2C
Lipoprotein(a) hyperlipoproteinemia: Progressive atherosclerotic cardiovascular disease	LA	II	1B
Malaria: Severe*	RBC exchange	III	2B
Multiple sclerosis
Acute attack/relapse	TPE	II	1A
Acute attack/relapse	IA	II	1B
Chronic	TPE	III	2B
Chronic	IA	III	2B
Myasthenia gravis
Acute, short-term treatment	TPE/IA	I	1B
Long-term treatment	TPE/IA	II	2B
Myeloma cast nephropathy	TPE	II	2B
Nephrogenic systemic fibrosis	ECP/TPE	III	2C
Neuromyelitis optica spectrum disorders (NMOSD)
Acute attack/relapse	TPE	II	1B
Acute attack/relapse	IA	II	1C
Maintenance	TPE	III	2C
N-methyl-D-aspartate receptor antibody encephalitis	TPE/IA	I	1C
Overdose, envenomation, and/or poisoning
Mushroom poisoning	TPE	II	2C
Envenomation	TPE	III	2C
Drug overdose/poisoning	TPE	III	2C
Paraneoplastic neurologic syndromes	TPE/IA	III	2C
Paraproteinemic demyelinating neuropathies; chronic acquired demyelinating polyneuropathies
Anti-myelin-associated glycoprotein (MAG) neuropathy	TPE	III	1C
IgA/IgG/IgM	TPE	I	1B
Multifocal motor neuropathy	TPE	IV	1C
Multiple myeloma	TPE	III	2C
Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS); Sydenham chorea
PANDAS, exacerbation	TPE	II	1B
Sydenham chorea, severe	TPE	III	2B
Pemphigus vulgaris: Severe	TPE	III	2B
Pemphigus vulgaris: Severe	ECP/IA	III	2C
Peripheral vascular diseases	LA	II	1B
Phytanic acid storage disease (Refsum disease)	TPE/LA	II	2C
Polycythemia vera; erythrocytosis
Polycythemia vera	Erythrocytapheresis	I	1B
Secondary erythrocytosis	Erythrocytapheresis	III	1C
Post-transfusion purpura (PTP)	TPE	III	2C
Progressive multifocal leukoencephalopathy (PML) associated with natalizumab	TPE	III	1C
Pruritus due to hepatobiliary disease: Treatment resistant	TPE	III	1C
Psoriasis: Disseminated pustular	ECP	III	2B
	Adsorptive cytapheresis	III	2C
	TPE	IV	2C
Red cell alloimmunization, prevention and treatment
Exposure to RhD-positive RBCs	RBC exchange	III	2C
Pregnancy, gestational age <20 weeks	TPE	III	2C
Scleroderma (systemic sclerosis)	TPE	III	2C
Scleroderma (systemic sclerosis)	ECP	III	2A
Sepsis with multiorgan failure	TPE	III	2B
Sickle cell disease, acute
Acute chest syndrome, severe	RBC exchange	II	1C
Acute stroke	RBC exchange	I	1C
Other complications	RBC exchange	III	2C
Sickle cell disease, non-acute
Stroke prophylaxis	RBC exchange	I	1A
Pregnancy	RBC exchange	II	2B
Recurrent vaso-occlusive pain	RBC exchange	II	2B
Pre-operative management	RBC exchange	III	2A
Steroid-responsive encephalopathy associated with autoimmune thyroiditis (Hashimoto encephalopathy)	TPE	II	2C
Stiff-person syndrome	TPE	III	2C
Sudden sensorineural hearing loss	LA/rheopheresis/TPE	III	2A
Systemic lupus erythematosus (SLE): Severe complications	TPE	II	2C
Thrombocytosis
Prophylactic or secondary	Thrombocytapheresis	III	2C
Symptomatic	Thrombocytapheresis	II	2C
Thrombotic microangiopathy
Coagulation-mediated: THBD, DGKE, and PLG mutations	TPE	III	2C
Complement-mediated: Complement factor gene mutations	TPE	III	2C
Complement-mediated: Factor H autoantibody	TPE	I	2C
Drug-associated: Clopidogrel	TPE	III	2B
Drug-associated: Gemcitabine/quinine	TPE	IV	2C
Drug-associated: Ticlopidine^¶	TPE	I	2B
Infection-associated: STEC-HUS, severe	TPE/IA	III	2C
Infection-associated: pHUS	TPE	III	2C
Thrombotic thrombocytopenic purpura (TTP; severe ADAMTS13 deficiency)	TPE	I	1A
Transplantation-associated	TPE	III	2C
Thyroid storm	TPE	II	2C
Toxic epidermal necrolysis (TEN): Refractory	TPE	III	2B
Transplantation, cardiac
Cellular/recurrent rejection	ECP	II	1B
Rejection prophylaxis	ECP	II	2A
Desensitization	TPE	II	1C
Antibody-mediated rejection	TPE	III	2C
Transplantation, hematopoietic stem cell, ABO incompatible (ABOi)
Major ABOi hematopoietic cells obtained from bone marrow	TPE	II	1B
Major ABOi hematopoietic cells obtained by apheresis	TPE	II	2B
Minor ABOi hematopoietic cells obtained by apheresis	RBC exchange	III	2C
Major/minor ABOi with pure RBC aplasia	TPE	III	2C
Transplantation, hematopoietic stem cell, HLA desensitization	TPE	III	2C
Transplantation, liver
Desensitization, ABOi living donor	TPE	I	1C
Desensitization, ABOi deceased donor/antibody-mediated rejection	TPE	III	2C
Desensitization, ABOi	ECP	III	2C
Acute rejection/immune suppression withdrawal	ECP	III	2B
Transplantation, lung
Bronchiolitis obliterans syndrome	ECP	II	1C
Antibody-mediated rejection/desensitization	TPE	III	2C
Transplantation, renal, ABO compatible
Antibody mediated rejection	TPE/IA	I	1B
Desensitization, living donor	TPE/IA	I	1B
Desensitization, deceased donor	TPE/IA	III	2C
Transplantation, renal, ABO incompatible
Desensitization, living donor	TPE/IA	I	1B
Antibody-mediated rejection	TPE/IA	II	1B
Vasculitis, ANCA-associated (AAV)
Microscopic polyangiitis (MPA)/granulomatous polyangiitis (GPA)/renal limited vasculitis (RLV): RPGN, Cr ≥5.7	TPE	I	1A
MPA/GPA/RLV: RPGN, Cr <5.7	TPE	III	2C
MPA/GPA/RLV: DAH	TPE	I	1C
Eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss)	TPE	III	2C
Vasculitis, IgA (Henoch-Schönlein purpura)
Crescentic RPGN	TPE	III	2C
Severe extrarenal manifestations	TPE	III	2C
Vasculitis, other
Behçet disease	Adsorptive cytapheresis	II	1C
Behçet disease	TPE	III	2C
Hepatitis B polyarteritis nodosa	TPE	II	2C
Idiopathic polyarteritis nodosa	TPE	IV	1B
Voltage-gated potassium channel (VGKC) antibody-related disease	TPE/IA	II	1B
Wilson disease, fulminant	TPE	I	1C

Refer to UpToDate topics on individual conditions for specific treatment recommendations. Due to UpToDate styling and alphabetization, the order of diseases in this table may differ slightly from the original source publication.

Category

Category I: Disorders for which apheresis is accepted as first-line therapy, either as a standalone treatment or in conjunction with other therapies.
Category II: Disorders for which apheresis is accepted as second-line therapy, either as a standalone treatment or in conjunction with other therapies.
Category III: Disorders for which the optimum role of apheresis therapy is not established.
Category IV: Disorders for which published evidence demonstrates or suggests apheresis to be ineffective or harmful.

Evidence

Evidence grade 1: Strong recommendation.
Evidence grade 2: Weak recommendation.
Evidence quality A: High-quality evidence.
Evidence quality B: Moderate-quality evidence.
Evidence quality C: Low-quality or very low-quality evidence.

TPE: therapeutic plasma exchange; IA: immunoadsorption; TPE-HV: high-volume therapeutic plasma exchange; ECP: extracorporeal photopheresis; DFPP: double filtration plasmapheresis; NYHA: New York Heart Association; RBC: red blood cell; LA: lipoprotein apheresis; Ig: immunoglobulin; STEC-HUS: Shiga toxin-producing E. Coli-associated (or other Shiga toxin-producing organism) hemolytic uremic syndrome; pHUS: post-transplant hemolytic uremic syndrome; HLA: human leukocyte antigen; RPGN: rapidly progressive glomerulonephritis; DAH: diffuse alveolar hemorrhage.

* UpToDate authors do not use RBC exchange for severe malaria.

¶ UpToDate authors consider this entity (thrombotic microangiopathy [TMA] in the setting of ticlopidine) to be immune thrombotic thrombocytopenic purpura (iTTP) rather than drug-induced TMA because the patients had severe ADAMTS13 deficiency.

From: Padmanabhan A, Connelly-Smith L, Aqui N, 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 Eighth Special Issue. J Clin Apher 2019; 34(3):171-354. https://onlinelibrary.wiley.com/doi/abs/10.1002/jca.21705. Copyright © 2019 Wiley Periodicals, Inc. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: permissions@wiley.com or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (https://onlinelibrary.wiley.com/).

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