Scully Marie
University College London Hospitals, London, UK.
Hematology. 2012 Apr;17 Suppl 1:S22-4. doi: 10.1179/102453312X13336169155178.
Thrombotic thrombocytopenic purpura (TTP) is an acute life threatening disorder, for which mortality remains relatively unchanged since the introduction of plasma therapy. Improved understanding of the pathophysiology has identified that the majority of cases result from antibodies directed against ADAMTS 13, which is required to cleave von Willebrand Factor. The use of monoclonal anti-CD 20 therapy removes IgG antibodies, resulting in increased ADAMTS 13 activity, improved time to remission and prevention of recurrent relapses. While further follow-up is required, the side effect profile of anti-CD 20 therapy appears improved compared to conventional immunosuppressive treatments. The use of ADAMTS 13 activity for monitoring can identify patients at risk of a TTP relapse and preemptive therapy with an anti-CD 20 can be considered.
血栓性血小板减少性紫癜（TTP）是一种急性危及生命的疾病，自采用血浆疗法以来，其死亡率相对保持不变。对病理生理学的深入了解表明，大多数病例是由针对ADAMTS 13的抗体引起的，ADAMTS 13是裂解血管性血友病因子所必需的。使用单克隆抗CD 20疗法可去除IgG抗体，从而提高ADAMTS 13活性，缩短缓解时间并预防复发。虽然需要进一步随访，但与传统免疫抑制治疗相比，抗CD 20疗法的副作用似乎有所改善。利用ADAMTS 13活性进行监测可以识别有TTP复发风险的患者，并可考虑采用抗CD 20进行预防性治疗。
