Kremer Hovinga Johanna A, Studt Jan-Dirk, Demarmels Biasiutti Franziska, Solenthaler Max, Alberio Lorenzo, Zwicky Caroline, Fontana Stefano, Taleghani Behrouz Mansouri, Tobler Andreas, Lämmle Bernhard
Central Hematology Laboratory, Inselspital, University of Bern, Bern, Switzerland.
Haematologica. 2004 Mar;89(3):320-4.
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.
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.
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.
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.
获得性血栓性血小板减少性紫癜（TTP）通常是由于自身抗体抑制ADAMTS - 13活性，导致超大血管性血友病因子多聚体的加工受损。TTP通常起病急骤，病程凶险，有时甚至致命。通过适当治疗，包括血浆置换和新鲜冰冻血浆置换，常辅以免疫抑制治疗，急性发作一般在数天至数周内缓解。
我们描述了3例获得性TTP患者的临床病程。1例对血浆置换难治，另外2例在该治疗后复发。所有3例均接受了脾切除术。监测了ADAMTS - 13活性和抑制剂水平。
所有3例患者最初的ADAMTS - 13活性均<5%。脾切除术后，2例患者针对ADAMTS - 13的抑制剂迅速消失，所有3例患者的ADAMTS - 13活性完全恢复。
对于因自身抗体介导的ADAMTS - 13缺乏而复发或对血浆置换难治的获得性TTP患者，脾切除术通过消除致病性自身抗体产生的来源，可能是一种成功的治疗方法。
