RECOVERY BY SPLENECTOMY IN PATIENTS WITH RELAPSED THROMBOTIC THROMBOCYTOPENIC PURPURA AND TREATMENT FAILURE TO PLASMA-EXCHANGE

Thrombotic thrombocytopenic purpura is a serious, potentially fatal di sease, and conventional plasma exchange appears to be the best initial therapy. Following this approach, survival in 90% of patients is avai lable. In patients with relapse and treatment failure to plasma exchan ge, splenectomy is recommended. The rationale for splenectomy and the relevant pathomechanisms involved are obscure. In the present paper tw o patients with TTP are reported who first responded to conventional t reatment strategies but later relapsed. Resumption of previous therapy was not able to continuously maintain normal platelet levels. Thus, s plenectomy was considered to be indicated. In contrast to former repor ts, repeated cycles of conventional plasma exchanges were performed un til 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 splenectom y. In addition no postoperative complications (bleeding, neurologic im pairment) have been observed. This favorable outcome might be due to t he strategy of repeated conventional plasma exchange procedures. The f ollow-up shows now event free disease for 2 years.