TY - JOUR AU - JA Kremer Hovinga, AU - JD Studt, AU - F Demarmels Biasiutti, AU - M Solenthaler, AU - L Alberio, AU - C Zwicky, AU - S Fontana, AU - BM Taleghani, AU - A Tobler, AU - B Laemmle, PY - 2004/03/01 Y2 - 2024/03/28 TI - Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura JF - Haematologica JA - haematol VL - 89 IS - 3 SE - Case Reports DO - 10.3324/%x UR - https://haematologica.org/article/view/3003 SP - 320-324 AB - BACKGROUND AND OBJECTIVES: Acquired thrombotic thrombocytopenic purpura (TTP) is often due to autoantibodies inhibiting ADAMTS-13 activity resulting in impaired processing of very large von Willebrand factor multimers. TTP usually presents with an acute onset and a fulminant, sometimes fatal course. With appropriate treatment including plasma exchange, and fresh frozen plasma replacement, often supplemented by immuno-suppressive therapy, the acute episode generally resolves within days to weeks. DESIGN AND METHODS: We describe the clinical course of 3 patients with acquired TTP. One was refractory to PE, the other 2 relapsed after this treatment. All three were treated with splenectomy. ADAMTS-13 activity and inhibitor levels were monitored. RESULTS: ADAMTS-13 activity was initially < 5% in all 3 patients. After splenectomy the inhibitor against ADAMTS-13 disappeared rapidly in 2 patients and there was full recovery of ADAMTS-13 activity in all 3 patients. INTERPRETATION AND CONCLUSIONS: Splenectomy, by eliminating a source of pathogenic autoantibody production, can be a successful treatment for patients with relapsing or plasma-refractory acquired TTP due to autoantibody-mediated ADAMTS-13 deficiency. ER -