title: Response to plasma exchange and steroids as combined therapy for patients with thrombotic thrombocytopenic purpura.
abstract: We describe our experience in the management of 11 consecutive patients with thrombotic thrombocytopenic purpura (TTP) treated with a combined therapy of plasma exchange (PE) and steroids. Nine patients (82%) achieved complete remission (CR) after a median of 6 rounds of PE (range 2-22). There were 3 early relapses managed in the same way as the initial episode. One patient relapsed 23 months after diagnosis achieving CR with standard therapy; another patient suffered several relapses, and splenectomy was performed after the last one. Three patients died, 2 of them with resistant disease 9 and 38 days after diagnosis, and the remaining one died due to AIDS-related complications while he was in CR. Eight patients are alive in CR with a median follow-up of 38 months (range 8-74). The combination of PE and steroids is a well-tolerated and effective treatment of TTP, but improvements in therapy are needed to manage refractory patients.
authors.affiliation: Haematology Department, University Hospital La Fe, Valencia, Spain. jrubia@san.gva.es
authors.forename: J
authors.initials: J
authors.lastname: de la Rubia
authors.forename: A
authors.initials: A
authors.lastname: López
authors.forename: F
authors.initials: F
authors.lastname: Arriaga
authors.forename: A R
authors.initials: AR
authors.lastname: Cid
authors.forename: A I
authors.initials: AI
authors.lastname: Vicente
authors.forename: M L
authors.initials: ML
authors.lastname: Marty
authors.forename: M A
authors.initials: MA
authors.lastname: Sanz
mesh_terms: D000328:Adult; D000368:Aged; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000401:mortality / Q000628:therapy*; D012008:Recurrence; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use*; D015996:Survival Rate
chemical_list: D013256:Steroids