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    Bortezomib for chronic relapsing thrombotic thrombocytopenic purpura: a case report

    发布于：2014年4月30日    文字：【大】【中】【小】

    Transfusion. 2014 Mar 24. doi: 10.1111/trf.12614. [Epub ahead of print]

    Bortezomib for chronic relapsing thrombotic thrombocytopenic purpura: a case report.

    Yates S1, Matevosyan K, Rutherford C, Shen YM, Sarode R.

    Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder characterized by a severe deficiency of ADAMTS13 activity. Although therapeutic plasma exchange (PLEX) is the standard of care, 30% to 50% patients develop exacerbation or relapse, requiring immunomodulatory agents. Of these agents, glucocorticoids, rituximab, and cyclosporine?A are the most frequently used.?We report a case of chronic relapsing TTP in a patient who had eight relapses over a 14-year period. After her seventh relapse, the patient demonstrated only partial response to glucocorticoids, two courses of rituximab, and cyclophosphamide. The eighth relapse occurred 58 days after her last PLEX and subsequent to this she received a course of bortezomib (Velcade, Millennium Pharmaceuticals, Inc.). After treatment with bortezomib the patient demonstrated a complete response with a progressive increase in ADAMTS13 activity from less than 5% to 22% accompanied by undetectable inhibitor, and she has remained PLEX free for more than 169 days.?Bortezomib may serve as an adjunct treatment in patients with acquired TTP who exhibit an incomplete response or are refractory to conventional management.

    获得性血栓性血小板减少性紫癜（TTP）是由于产生抗ADAMTS-13自身抗体，其治疗包括血浆置换，并加用糖皮质激素和免疫抑制剂（主要是环磷酰胺，其他包括环孢素A、长春新碱）以抑制自身抗体产生，美罗华（CD20单抗）也部分有效；最近万珂在难治性获得性TTP开始逐渐有病例报道。

    本文再报道了一例获得性TTP患者，48岁，女性，在14年内反复复发8次，最后血浆置换依赖，糖皮质激素+环磷酰胺+美罗华效果不佳；考虑美罗华是针对的是B细胞，最后使用万珂（bortezomib，一种蛋白酶体抑制剂，可以诱导B细胞和浆细胞凋亡，最主要用于多发性骨髓瘤的化疗）以希望减少浆细胞（免疫球蛋白的直接产生细胞），病情明显好转，同时未再需要血浆置换；该患者在使用万珂过程中经历了粒细胞缺乏、败血症、肺间质纤维化不良反应。

    http://www.ncbi.nlm.nih.gov/pubmed/24655327