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[NEJM医学影像]: 血栓性血小板减少紫癜
2019年04月30日 临床影像, 临床话题 暂无评论

IMAGES IN CLINICAL MEDICINE

Thrombotic Thrombocytopenic Purpura

Shruti Bhandari and Rohit Kumar

N Engl J Med 2019; 380:e23
DOI: 10.1056/NEJMicm1813768

A 35-year-old man presented to the emergency department with a 2-day history of abdominal pain, headache, and brown urine. On examination, his temperature was 37.5°C. He was alert and oriented to time, place, and self, and he had no purpura or petechiae. Laboratory evaluation revealed a hemoglobin level of 8.6 g per deciliter (normal range, 13.7 to 17.5) and a platelet count of 6000 per cubic millimeter (normal range, 140,000 to 370,000). Levels of indirect bilirubin and lactate dehydrogenase were elevated, haptoglobin was undetectable, and the serum creatinine level was 1 mg per deciliter (88 μmol per liter; normal range, 0.6 to 1.3 mg per deciliter [57 to 115 μmol per liter]). Test results for infection with the human immunodeficiency virus were negative. A peripheral-blood smear showed numerous schistocytes (arrows). These findings were consistent with a microangiopathic hemolytic anemia. Plasma exchange for suspected thrombotic thrombocytopenic purpura was started immediately. Without the prompt initiation of treatment, mortality from thrombotic thrombocytopenic purpura is high. The patient underwent plasma exchange and received intravenous methylprednisolone for 5 days and had resolution of symptoms and an increased platelet count. The diagnosis was confirmed 2 days after initiation of treatment, with ADAMTS13 activity of 1%. He was discharged with instructions for tapering glucocorticoid treatment. Three months after discharge, he remained well.

一名35岁男性患者因腹痛、头痛及棕色尿2天到急诊就诊。检查发现,患者体温37.5°C,神志清楚,具有时间、地点和人物定向力,无紫癜或淤斑。实验室检查,血红蛋白 8.6 g/dl(正常范围13.7 - 17.5),血小板计数 6000/ml(正常范围140,000 - 370,000)。间接胆红素和乳酸脱氢酶水平升高,结合珠蛋白无法测到,血清肌酐水平1 mg/dl(88 μmol/L;正常范围,0.6 - 1.3 mg/dl [57 - 115 μmol/L])。HIV检查结果阴性。外周血涂片显示大量破碎红细胞(箭头)。这些表现符合微血管病性溶血性贫血。因怀疑为血栓性血小板减少性紫癜,立即行血浆交换。如果不立即开始治疗,血栓性血小板减少性紫癜的病死率很高。患者接受了血浆交换及静脉甲基强的松龙治疗5天,症状缓解,血小板计数升高。开始治疗2天后,ADAMTS13检测结果为1%,因此确诊为血栓性血小板减少性紫癜。患者接受有关糖皮质激素减量治疗的指导后出院。出院后3个月,患者一般情况良好。

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