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©2013 UpToDate ® Print Email American Society for Apheresis 2010 indication categories for therapeutic apheresis Modality: Therapeutic plasma exchange; Category: I Acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome) ANCA-associated rapidly progressive glomerulonephritis (Granulomatosis with polyangiitis [Wegener's]) Dialysis dependence Diffuse alveolar hemorrhage Anti-glomerular basement membrane disease (Goodpasture's syndrome) Dialysis independence Diffuse alveolar hemorrhage Chronic inflammatory demyelinating polyradiculoneuropathy Cryoglobulinemia Severe/symptomatic Focal segmental glomerulosclerosis recurrent Hemolytic-uremic syndrome Atypical HUS due to autoantibody to factor H Hyperviscosity in monoclonal gammopathies Treatment of symptoms Prophylaxis for rituximab Myasthenia gravis Moderate-severe Pre-thymectomy Paraproteinemic polyneuropathies IgG/IgA IgM Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) (exacerbation) Renal transplantation Antibody mediated rejection Sydenham's chorea Thrombotic microangiopathy: drug-associated Ticlopidine/clopidogrel Thrombotic thrombocytopenic purpura Wilson's disease, fulminant hepatic failure with hemolysis Modality: Therapeutic plasma exchange; Category: II ABO incompatible hematopoietic cell transplantation HPC, marrow HPC, apheresis ABO incompatible solid organ transplantation Kidney Heart (<40 months of age) Acute disseminated encephalomyelitis Autoimmune hemolytic anemia Cold agglutinin disease (life-threatening) Catastrophic antiphospholipid syndrome Chronic focal encéphalites (Rasmussen's encéphalites) Familial hypercholesterolemia Homozygotes with small blood volume Hemolytic-uremic syndrome Atypical HUS due to complement factor gene mutations Lambert-Eaton myasthenic syndrome Multiple sclerosis Acute CNS inflammatory demyelinating disease unresponsive to steroids Myeloma cast nephropathy Neuromyelitis optica (Devic's syndrome) Overdose, venoms, and poisoning Mushroom poisoning Phytanic acid storage disease (Refsum's disease) Pure red cell aplasia Red cell alloimmunization in pregnancy, before intrauterine transfusion availability Renal transplantation Desensitization, living donor, positive crossmatch due to donor specific HLA antibody Systemic lupus erythematosus, severe (eg, cerebritis, diffuse alveolar hemorrhage) Modality: Therapeutic plasma exchange; Category: III ABO incompatible solid organ transplantation Liver perioperative Acute liver failure

Red cell alloimmunization in pregnancy, before intrauterine transfusion availability Renal transplantation Desensitization, living donor, positive crossmatch due to donor specific HLA antibody Systemic lupus erythematosus, severe (eg, cerebritis, diffuse alveolar hemorrhage) Modality: Therapeutic plasma exchange; Category: III ABO incompatible solid organ transplantation Liver perioperative Acute liver failure ANCA-associated rapidly progressive glomerulonephritis (Wegener's Granulomatosis) Dialysis independence Aplastic anemia Autoimmune hemolytic anemia Warm autoimmune hemolytic anemia Cardiac allograft rejection Treatment of antibody mediated rejection Dilated cardiomyopathy NYHA II-IV Hypertriglyceridemic pancreatitis Immune complex rapidly progressive glomerulonephritis Multiple sclerosis Chronic progressive Nephrogenic systemic fibrosis Overdose, venoms, and poisoning Envenomation Monoclonal antibody with PML Other compounds Paraneoplastic neurologic syndromes Paraproteinemic polyneuropathies Multiple myeloma Post-transfusion purpura Renal transplantation High PRA, cadaveric donor Scleroderma (progressive systemic sclerosis) Sepsis with multiorgan failure Thrombotic microangiopathy: drug-associated Cyclosporine/tacrolimus Thrombotic microangiopathy: hematopoietic stem cell transplant-associated Thyroid storm Modality: Rheopheresis; Category: III Age-related macular degeneration, dry Modality: RBC exchange; Category: I Babesiosis Severe Sickle cell disease Acute stroke Modality: RBC exchange; Category: II Babesiosis High-risk population Malaria, severe Sickle cell disease Acute chest syndrome Prophylaxis for primary or secondary stroke; prevention of transfusional iron overload Modality: RBC exchange; Category: III Sickle cell disease Multi-organ failure Modality: Extracorporeal photopheresis; Category: I Cardiac allograft rejection Prophylaxis Cutaneous T cell lymphoma; mycosis fungoides; Sézary syndrome Erythrodermic Modality: Extracorporeal photopheresis; Category: II Cardiac allograft rejection Treatment of rejection Graft-versus-host disease Skin (chronic) Skin (acute) Lung allograft rejection Modality: Extracorporeal photopheresis; Category: III Graft-versus-host disease Non-skin (acute/chronic) Cutaneous T cell lymphoma; mycosis fungoides; Sézary syndrome Non-erythrodermic Nephrogenic systemic fibrosis Pemphigus vulgaris Modality: Immune adsorption; Category: II Chronic focal encéphalites (Rasmussen's encéphalites) Cryoglobulinemia Secondary to hepatitis C virus Rheumatoid arthritis, refractory Modality: Immune adsorption; Category: III

Cutaneous T cell lymphoma; mycosis fungoides; Sézary syndrome Non-erythrodermic Nephrogenic systemic fibrosis Pemphigus vulgaris Modality: Immune adsorption; Category: II Chronic focal encéphalites (Rasmussen's encéphalites) Cryoglobulinemia Secondary to hepatitis C virus Rheumatoid arthritis, refractory Modality: Immune adsorption; Category: III Coagulation factor inhibitors Dilated cardiomyopathy NYHA II-IV Paraneoplastic neurologic syndromes Paraproteinemic polyneuropathies IgG/IgA or IgM Modality: Selective removal; Category: I Familial hypercholesterolemia Homozygotes Modality: Selective removal; Category: II Familial hypercholesterolemia Heterozygotes Modality: Erythrocytapheresis; Category: III Hereditary hemochromatosis Polycythemia vera and erythrocytosis Polycythemia vera Secondary erythrocytosis Modality: Leukocytapheresis; Category: I Hyperleukocytosis Leukostasis Modality: Leukocytapheresis; Category: III Hyperleukocytosis Prophylaxis Modality: Adsorptive cytapheresis; Category: II Inflammatory bowel disease Modality: Thrombocytapheresis; Category: II Thrombocytosis Symptomatic Modality: Thrombocytapheresis; Category: III Thrombocytosis Prophylactic or secondary Disease entities listed under category IV (see topic text) have been omitted from the table. Category I: Disorders for which apheresis is accepted as first-line therapy. Category II: Disorders for which apheresis is accepted as second-line therapy. 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. ANCA: anti-neutrophil cytoplasmic autoantibody; CNS: central nervous system; HLA: human leukocyte antigen; HPC: hematopoietic progenitor cell; HCT; hematopoietic stem cell transplantation; HUS: hemolytic-uremic syndrome; NYHA: New York Heart Association; PANDAS: pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections; PML: progressive multifocal leukoencephalopathy; PRA: panel reactive antibody. Modified from: 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; 25:83.