Article: article from journal or magazin.
Plasma therapy in thrombotic thrombocytopenic purpura: review of the literature and the Bern experience in a subgroup of patients with severe acquired ADAMTS-13 deficiency.
Seminars in Hematology
Publication types: Journal Article Publication Status: ppublish
Based on clinical studies daily plasma exchange (PE) has become the first-choice therapy for thrombotic thrombocytopenic purpura (TTP) since 1991. Recent findings may explain its effectiveness, which particularly may include supply of ADAMTS-13 and removal of anti-ADAMTS-13 autoantibodies and unusually large von Willebrand factor (VWF) multimers. The most preferable PE regimens as well as replacement fluids are discussed and treatment-related adverse reactions are summarized. Proposals for a potential reduction of their frequency and for improvement of treatment efficiency are given. These suggestions are partially based on the experience of our institution in adult patients with severe ADAMTS-13 deficiency (<5% activity), and include (1) continuous calcium-gluconate infusion during PE in order to reduce citrate-related adverse reactions; (2) the evaluation of solvent/detergent-treated (S/D) plasma as replacement fluid in order to reduce adverse events due to fresh frozen plasma (FFP); (3) the evaluation of immunoadsorption in order to increase procedural efficiency in autoantibody removal; and (4) the substitution of ADAMTS-13 by means of recombinant drug instead of plasma.
ADAM Proteins, Adolescent, Adult, Aged, Blood Platelets/drug effects, Female, Follow-Up Studies, Humans, Male, Metalloendopeptidases/antagonists & inhibitors, Metalloendopeptidases/deficiency, Middle Aged, Plasma Exchange/adverse effects, Plasma Exchange/methods, Protease Inhibitors/pharmacology, Purpura, Thrombotic Thrombocytopenic/blood, Purpura, Thrombotic Thrombocytopenic/therapy, Retrospective Studies
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