title: 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.
abstract: 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.
authors.affiliation: Central Hematology Laboratory, Inselspital, University Hospital, Bern, Switzerland.
authors.forename: Stefano
authors.initials: S
authors.lastname: Fontana
authors.forename: Johanna A
authors.initials: JA
authors.lastname: Kremer Hovinga
authors.forename: Jan-Dirk
authors.initials: JD
authors.lastname: Studt
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Alberio
authors.forename: Bernhard
authors.initials: B
authors.lastname: Lämmle
authors.forename: Behrouz Mansouri
authors.initials: BM
authors.lastname: Taleghani
mesh_terms: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000368:Aged; D001792:Blood Platelets / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008666:Metalloendopeptidases / Q000037:antagonists & inhibitors / Q000172:deficiency* / Q000378:metabolism; D008875:Middle Aged; D010951:Plasma Exchange / Q000009:adverse effects / Q000379:methods*; D011480:Protease Inhibitors / Q000494:pharmacology; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000628:therapy*; D012189:Retrospective Studies
chemical_list: D011480:Protease Inhibitors; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human