Recovery by splenectomy in patients with relapsed thrombotic thrombocytopenic purpura and treatment failure to plasma exchange

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 r...

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Published inSeminars in thrombosis and hemostasis Vol. 21; no. 2; p. 161
Main Authors Höffkes, H G, Weber, F, Uppenkamp, M, Meusers, P, Teschendorf, C, Philipp, T, Brittinger, G
Format Journal Article
LanguageEnglish
Published United States 01.01.1995
Subjects
Adrenal Cortex Hormones - therapeutic use
Adult
Combined Modality Therapy
Female
Follow-Up Studies
Humans
Male
Plasma Exchange
Platelet Aggregation Inhibitors - therapeutic use
Purpura, Thrombotic Thrombocytopenic - pathology
Purpura, Thrombotic Thrombocytopenic - surgery
Recurrence
Splenectomy
Treatment Failure
Treatment Outcome
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Summary: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.
ISSN:0094-6176
1098-9064
DOI:10.1055/s-2007-1000391