title: Recovery by splenectomy in patients with relapsed thrombotic thrombocytopenic purpura and treatment failure to plasma exchange.
abstract: Thrombotic thrombocytopenic purpura is a serious, potentially fatal disease, and conventional plasma exchange appears to be the best initial therapy. Following this approach, survival in 90% of patients is available. In patients with relapse and treatment failure to plasma exchange, splenectomy is recommended. The rationale for splenectomy and the relevant pathomechanisms involved are obscure. In the present paper two patients with TTP are reported who first responded to conventional treatment strategies but later relapsed. Resumption of previous therapy was not able to continuously maintain normal platelet levels. Thus, splenectomy was considered to be indicated. In contrast to former reports, repeated cycles of conventional plasma exchanges were performed until a transient steady state (12 hrs) of the platelet counts occurred. Then splenectomy was performed immediately and, in contrast to former reports, no reinstitution of treatment was necessary after splenectomy. In addition no postoperative complications (bleeding, neurologic impairment) have been observed. This favorable outcome might be due to the strategy of repeated conventional plasma exchange procedures. The follow-up shows now event free disease for 2 years.
authors.affiliation: Division of Hematology, University of Essen, Germany.
authors.forename: H G
authors.initials: HG
authors.lastname: Höffkes
authors.forename: F
authors.initials: F
authors.lastname: Weber
authors.forename: M
authors.initials: M
authors.lastname: Uppenkamp
authors.forename: P
authors.initials: P
authors.lastname: Meusers
authors.forename: C
authors.initials: C
authors.lastname: Teschendorf
authors.forename: T
authors.initials: T
authors.lastname: Philipp
authors.forename: G
authors.initials: G
authors.lastname: Brittinger
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D010951:Plasma Exchange; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D011697:Purpura, Thrombotic Thrombocytopenic / Q000473:pathology / Q000601:surgery*; D012008:Recurrence; D013156:Splenectomy; D017211:Treatment Failure; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D010975:Platelet Aggregation Inhibitors