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Abstract：
Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially life-threatening hematologic disorder characterized by hemolytic anemia, thrombocytopenia, renal failure, fever, and neurologic dysfunction. While cases often do not present with all five characteristics (<5%), TTP can be hereditary or acquired, often due to a deficiency or dysfunction of the ADAMST13 enzyme. Here, we describe a case of infection-induced acquired TTP in a middle-aged male with urinary tract infection (UTI) and perianal abscess. Suspicion arose from hematologic abnormalities, fever, thrombocytopenia, acute renal failure, and the presence of an underlying infection. A PLASMIC score of 6 (indicating a 72% probability of ADAMTS13 deficiency) prompted ADAMTS13 level testing, revealing levels <5% with the presence of an inhibitor, confirming TTP diagnosis. Treatment with high-dose steroids and daily plasma exchange yielded a swift platelet response, necessitating only two to three days of plasma exchange. In addition, incision and drainage of the perianal abscess were performed. The patient was discharged on daily prednisone and initiated on four doses of weekly Rituximab to mitigate recurrence risk. This case underscores the importance of early suspicion and treatment in infectious triggers such as UTI/perianal abscess, offering crucial diagnostic and prognostic insights.
摘要：
血栓性血小板减少性紫癜（TTP）是一种罕见但可能危及生命的血液病，以溶血性贫血为特征，血小板减少症,肾功能衰竭,发烧,和神经功能障碍.虽然病例通常不具备所有五个特征(<5%)，TTP可以是遗传性的或获得性的，通常由于ADAMST13酶的缺乏或功能障碍。这里,我们描述了一例中老年男性尿路感染（UTI）和肛周脓肿感染引起的获得性TTP。怀疑是由血液学异常引起的，发烧,血小板减少症,急性肾功能衰竭,以及潜在感染的存在。PLASMIC得分为6（表明ADAMTS13缺陷的概率为72％）提示ADAMTS13水平测试，在存在抑制剂的情况下揭示<5%的水平，确认TTP诊断。高剂量类固醇和每日血浆置换治疗可产生快速的血小板反应，只需要两到三天的血浆交换。此外,对肛周脓肿进行切开引流。患者每天使用泼尼松出院，并开始每周使用四剂利妥昔单抗以减轻复发风险。该病例强调了早期怀疑和治疗感染性诱因如UTI/肛周脓肿的重要性，提供关键的诊断和预后见解。
