Verbeke Len, Delforge Michel, Dierickx Daan
University Hospitals Gasthuisberg, Department of Internal Medicine, Leuven, Belgium.
Blood Coagul Fibrinolysis. 2010 Jan;21(1):3-10. doi: 10.1097/MBC.0b013e32833335eb.
During the last three decades knowledge regarding the pathophysiology of thrombotic thrombocytopenic purpura (TTP) has dramatically increased. The discovery of ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13) deficiency in a subset of patients with TTP has been an important milestone. Apart from this, the use of therapeutic plasma exchange has reduced mortality rates in TTP from 80-90% to 10-20%. Nevertheless, TTP remains a possibly lethal disorder, in which early recognition of symptoms remains extremely important. In the last few years some interesting new insights into TTP have arisen. Firstly, promising reports on rituximab in the treatment of refractory and relapsing cases of TTP have been published. Secondly, risk stratification by means of ADAMTS13 deficiency and ADAMTS13 antibodies might lead to a more tailored approach in treating TTP patients.
在过去三十年里，关于血栓性血小板减少性紫癜（TTP）病理生理学的知识有了显著增加。在一部分TTP患者中发现了ADAMTS13（一种具有血小板反应蛋白基序的解整合素样金属蛋白酶13）缺乏，这是一个重要的里程碑。除此之外，治疗性血浆置换的应用已将TTP的死亡率从80%-90%降至10%-20%。然而，TTP仍然是一种可能致命的疾病，早期识别症状仍然极为重要。在过去几年里，对TTP有了一些有趣的新见解。首先，关于利妥昔单抗治疗难治性和复发性TTP病例的前景良好的报告已发表。其次，通过ADAMTS13缺乏和ADAMTS13抗体进行风险分层可能会导致在治疗TTP患者时采取更具针对性的方法。
