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A case of thrombotic thrombocytopenic purpura induced by acute pancreatitis

机译:急性胰腺炎引起的血栓性血小板减少性紫癜一例

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摘要

Thrombotic thrombocytopenic purpura (TTP) is a multisystemic microvascular disorder that may be caused by an imbalance between unusually large von Willebrand factor multimers and the cleaving protease ADAMTS13. In acquired TTP, especially in secondary TTP with various underlying diseases, the diagnosis is difficult because there are many cases that do not exhibit severe deficiency of ADAMTS13 or raised levels of ADAMST13 inhibitors. It is well known that collagen disease, malignancy, and hematopoietic stem cell transplantation can be underlying conditions that induce TTP. However, TTP induced by acute pancreatitis, as experienced by our patient, has rarely been reported. Our patient completely recovered with treatments using steroids and plasma exchange (PE) only. In cases where patients develop acute pancreatitis with no apparent causes for hemolytic anemia and thrombocytopenia, the possibility of TTP should be considered. Treatments for TTP including PE should be evaluated as soon as a diagnosis is made.
机译:血栓性血小板减少性紫癜(TTP)是一种多系统性微血管疾病,可能是由异常大的von Willebrand因子多聚体与裂解蛋白酶ADAMTS13之间的不平衡引起的。在获得性TTP中,尤其是在具有各种潜在疾病的继发性TTP中,由于许多病例并未表现出ADAMTS13的严重缺乏或ADAMST13抑制剂水平升高,因此诊断很困难。众所周知,胶原蛋白疾病,恶性肿瘤和造血干细胞移植可能是诱发TTP的潜在条件。然而,正如我们的患者所经历的那样,由急性胰腺炎引起的TTP很少被报道。我们的患者仅使用类固醇和血浆置换(PE)进行治疗即可完全康复。如果患者发展为急性胰腺炎,没有明显原因导致溶血性贫血和血小板减少,应考虑使用TTP的可能性。诊断后应立即评估包括PE在内的TTP的治疗方法。

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