American Society for Apheresis 2023 indications for therapeutic apheresis and cytapheresis procedures

Indication	Modality	Category	Evidence
Acute disseminated encephalomyelitis (ADEM): Steroid refractory	TPE	II	2C
Acute fatty liver of pregnancy	TPE	III	2B
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	DFPP	III	2B
Alzheimer disease, mild or moderate	TPE	III	2A
Amyloidosis, systemic, dialysis-related	Beta2 microglobulin adsorption	II	2B
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 dermatitis (atopic eczema), recalcitrant	ECP/IA/TPE/DFPP	III	2B
Autoimmune dysautonomia	TPE	III	2C
Autoimmune hemolytic anemia (AIHA), severe
Severe cold agglutinin disease	TPE	II	2C
Severe warm AIHA	TPE	III	2C
Babesiosis, severe	RBC exchange	III	2C
Burn shock resuscitation	TPE	III	2B
Cardiac neonatal lupus	TPE	III	2C
Catastrophic antiphospholipid syndrome (CAPS)	TPE	I	2C
Chronic acquired demyelinating polyneuropathies
IgG/IgA/IgM related	TPE	I	1B
Anti-myelin-associated glycoprotein	TPE	III	1C
Chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins, and disialosyl antibodies (CANOMAD)/Chronic ataxic neuropathy with disialosyl antibodies syndrome (CANDA)	TPE	III	2C
Chronic focal encephalitis (Rasmussen encephalitis)	TPE/IA	III	2C
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)	TPE/IA	I	1B
Coagulation factor deficiency and inhibitors	IA	III	2B
Coagulation factor deficiency and inhibitors	TPE	III	2C
Complex regional pain syndrome, chronic	TPE	III	2C
Cryoglobulinemia, severe/symptomatic	TPE/DFPP	II	2A
Cryoglobulinemia, severe/symptomatic	IA	II	2B
Cutaneous T cell lymphoma (CTCL)
Erythrodermic mycosis fungoides/Sézary syndrome	ECP	I	1B
Non-erythrodermic mycoses fungoides	ECP	III	2B
Dilated cardiomyopathy, idiopathic, NYHA II-IV	IA	II	1B
Dilated cardiomyopathy, idiopathic, NYHA II-IV	TPE	III	2C
Erythrocytosis
Polycythemia vera	Erythrocytapheresis	I	1B
Secondary erythrocytosis	Erythrocytapheresis	III	1C
Erythropoietic protoporphyria, liver disease	TPE/RBC exchange	II	2C
Familial hypercholesterolemia
Homozygous individuals	LA	I	1A
Heterozygous individuals	LA	II	1A
All patients	TPE	II	1B
Focal segmental glomerulosclerosis (FSGS)
Recurrent in kidney transplant	TPE/IA	I	1B
All types	LA	II	2C
Steroid resistant in native kidney	TPE	III	2C
Graft-versus-host disease (GVHD)
Acute	ECP	II	1B
Chronic	ECP	II	1B
Hemophagocytic lymphocytosis (HLH)	TPE	III	2C
Heparin-induced thrombocytopenia and thrombosis
Pre-procedure	TPE/IA	III	2C
Refractory or with thrombosis	TPE	III	2C
Hereditary hemochromatosis	Erythrocytapheresis	I	1B
Hyperleukocytosis	Leukocytapheresis	III	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
Idiopathic inflammatory myopathies
Anti-synthetase-syndrome	TPE	III	2B
Clinically amyopathic dermatomyositis	TPE	III	2B
Immune-mediated necrotizing myopathies	TPE	III	2B
IgA nephropathy (Berger's disease)
Chronic progressive	TPE	III	2C
Crescentic	TPE	III	2B
Immune checkpoint inhibitors, immune-related adverse events	TPE	III	2C
Immune thrombocytopenia (ITP), refractory	TPE/IA	III	2C
Inflammatory bowel disease
Ulcerative colitis	Adsorptive cytapheresis	II	1B
Crohn disease	Adsorptive cytapheresis/ECP	III	1B/2C
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/IA	II	1A/1B
Chronic	TPE/IA	III	2B
Myasthenia gravis
Acute, short-term treatment	TPE/DFPP/IA	I	1B
Long-term treatment	TPE/DFPP/IA	II	2B
Myeloma cast nephropathy	TPE	II	2B
Nephrogenic systemic fibrosis	ECP/TPE	III	2C
Neuromyelitis optical spectrum disorders (NMOSD)
Acute attack/relapse	TPE/IA	II	1B/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
Other	TPE/RBC exchange	III	2C
Paraneoplastic autoimmune retinopathies	TPE	III	2C
Paraneoplastic neurologic syndromes	TPE/IA	III	2C
Pediatric autoimmune neuropsychiatric disorders
Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci (PANDAS)/Pediatric acute-onset neuropsychiatric syndrome (PANS), exacerbation	TPE	II	1B
Sydenham chorea, severe	TPE	III	2B
Pemphigus vulgaris, severe	TPE	III	2B
Pemphigus vulgaris, severe	IA/ECP/DFPP	III	2C
Peripheral vascular diseases	LA	II	1B
Phytanic acid storage disease (Refsum disease)	TPE/LA	II	2C
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 blood cell alloimmunization, pregnancy complications
Hemolytic disease of the fetus and newborn	TPE	III	2C
RhD alloimmunization prophylaxis after transfusion	RBC exchange	IV	2C
Sepsis with multiorgan failure	TPE	III	2A
Sickle cell disease
Acute stroke	RBC exchange	I	1C
Acute chest syndrome, severe	RBC exchange	II	1C
Other acute complications	RBC exchange/TPE	III	2C
Stroke prophylaxis	RBC exchange	I	1A
Pregnancy	RBC exchange	II	2B
Recurrent vaso-occlusive pain	RBC exchange	II	2B
Preoperative 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/DFPP/TPE	III	2A
Systemic lupus erythematosus (SLE): Severe complications	TPE	II	2C
Systemic sclerosis	ECP	III	2A
Systemic sclerosis	TPE	III	2C
Thrombocytosis
Symptomatic	Thrombocytapheresis	II	2C
Prophylactic or secondary	Thrombocytapheresis	III	2C
Thrombotic microangiopathy
Coagulation-mediated, due to pathogenic variants in THBD, DGKE, or PLG	TPE	III	2C
Complement-mediated, due to factor H autoantibodies	TPE	I	2C
Complement-mediated, due to pathogenic variants in complement regulatory genes	TPE	III	2C
Drug-associated: Ticlopidine^¶	TPE	I	2B
Drug-associated: Clopidogrel	TPE	III	2B
Drug-associated: Gemcitabine	TPE	IV	2C
Drug-associated: Quinine	TPE	IV	2C
Infection-associated, from Shiga toxin-producing Escherichia coli (STEC-HUS), severe	TPE/IA	III	2C
Infection-associated, from Streptococcus pneumoniae (pHUS)	TPE	III	2C
Pregnancy associated, severe	TPE	III	2C
Pregnancy associated, extremely preterm preeclampsia, severe	TPE/LA	III	2C
Thrombotic thrombocytopenic purpura (TTP; immune, with ADAMTS13 deficiency)	TPE	I	1A
Transplantation-associated	TPE	III	2C
Thyroid storm	TPE	II	2C
Toxic epidermal necrolysis (TEN), refractory	TPE	III	2B
Transplantation, heart
Cellular/recurrent rejection	ECP	II	1B
Rejection prophylaxis	ECP/TPE	II	2A/IC
Desensitization	TPE	II	1C
Antibody-mediated rejection	TPE	III	2C
Transplantation, hematopoietic stem cell, ABO incompatible
Major ABO incompatible, hematopoietic cells obtained from bone marrow	TPE	II	1B
Major ABO incompatible, hematopoietic cells obtained by apheresis	TPE	II	2B
Minor ABO incompatible, hematopoietic cells obtained by apheresis	RBC exchange	III	2C
Pure RBC aplasia	TPE	III	2C
Transplantation, hematopoietic stem cell, HLA desensitization	TPE	III	2C
Transplantation, intestine
Antibody mediated rejection	TPE	III	2C
Desensitization	TPE	III	2C
Transplantation, kidney, ABO compatible
Antibody mediated rejection	TPE/IA	I	1B
Desensitization/prophylaxis, living donor	TPE/IA	I	1B
Transplantation, kidney, ABO incompatible
Desensitization, living donor	TPE/IA	I	1B
Antibody-mediated rejection	TPE/IA	II	1B
Transplantation, liver
Desensitization, ABO incompatible, living donor	TPE	I	1C
Desensitization, ABO incompatible, deceased donor	TPE	III	2C
Antibody-mediated rejection	ECP/TPE	III	2B/2C
Immune suppression withdrawal	ECP	III	2B
Desensitization, ABO incompatible	ECP	III	2C
Transplantation, lung
Chronic allograft dysfunction	ECP	II	1C
Bronchiolitis obliterans syndrome	ECP	II	1C
Antibody-mediated rejection/desensitization	TPE	III	2C
Desensitization	TPE	III	2C
Vaccine-induced thrombotic thrombocytopenia, refractory	TPE	III	2C
Vasculitis, ANCA-associated
Microscopic polyangiitis	TPE	III	1B
Granulomatosis with polyangiitis	TPE	III	1B
Eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss)	TPE	III	2C
Vasculitis, IgA (Henoch-Schönlein purpura)
Crescentic rapidly progressive glomerulonephritis (RPGN)	TPE	III	2C
Severe extrarenal manifestations	TPE	III	2C
Vasculitis, other
Hepatitis B polyarteritis nodosa	TPE	II	2C
Kawasaki disease	TPE	III	2C
Multisystem inflammatory syndrome in children (MIS-C)	TPE	III	2C
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 optimal 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; HLA: human leukocyte antigen.

* 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: Connelly-Smith L, Alquist CR, Aqui NA, 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 Ninth Special Issue. J Clin Apher 2023; 38:77. https://onlinelibrary.wiley.com/doi/10.1002/jca.22043. Copyright © 2023 Wiley Periodicals LLC. 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: [email protected] 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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American Society for Apheresis 2023 indications for therapeutic apheresis and cytapheresis procedures