[Therapeutic apheresis in first-line treatment of hematological disorders - an evidence-based review]

Therapeutic apheresis (TA) is an important treatment for certain conditions. TA refers to various procedures in which either plasma (plasmapheresis) or specific blood cell fractions (cyta-pheresis) are removed. These include, among others, extracorporeal photopheresis (EP), erythrocytapheresis (EA), therapeutic plasma exchange (TPE), and therapeutic erythrocyte exchange (TEE). The indications for these procedures in specific diseases are published by the American Society for Apheresis (ASFA) as well as by hematology societies such as the American Society of Hematology (ASH) and the British Society of Haematology (BSH). There are only a few indications for therapeutic apheresis as first-line therapy for hematologic diseases, either as primary treatment or in combination with other therapies, for which strong evidence (Grade 1A-1C) exists. These indications include TPE for symptomatic hyperviscosity syndrome, hyperviscosity syndrome prophylaxis during rituximab therapy for Waldenström's macroglobulinemia, TEE for acute complications of sickle cell disease such as stroke and for the prophylaxis of such events, as well as the use of TPE for thrombotic thrombocytopenic purpura. Less well-known indications with strong evidence include the treatment of erythrocytosis in polycythemia vera and the treatment of iron overload in hereditary hemochromatosis using EA. Similarly, EP is an established treatment option for mycosis fungoides and Sézary syndrome. The aim of this paper is to summarize these indications.