Kunze A, Terborg C, Gerth J, Heide G, Witte O W
Neurologische Klinik der Universität Jena, Jena, Deutschland.
Nervenarzt. 2004 Aug;75(8):785-9. doi: 10.1007/s00115-004-1682-y.
Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy. Besides anemia and thrombocytopenia, neurological impairment is common in TTP. A 42-year-old woman was admitted to a department of obstetrics/gynecology because of severe vaginal bleeding due to thrombocytopenia. After platelet transfusion, the patient developed a reduced level of consciousness, confusion, headache, and fever. CT scan did not show pathological changes. Transcranial Doppler sonography revealed increased blood flow velocities of all basal cerebral arteries. Because encephalitis was suspected the patient was transferred to the neurological department. CSF and cerebral magnetic resonance imaging studies were normal. Finally, the detection of schistocytes in the peripheral blood smear and the strong elevation of LDH led to the diagnosis of TTP. After plasma exchange over 3 consecutive days the patient achieved complete remission. The diagnosis was confirmed by laboratory tests (activity of ADAMTS13 <5%, IgG antibodies against ADAMTS13). Platelet transfusion may adversely affect the outcome of patients with suspected TTP. Severely deficient activity of the von Willebrand factor cleaving protease (ADAMTS13) is specific for thrombotic thrombocytopenic purpura.
血栓性血小板减少性紫癜（TTP）是一种罕见的血栓性微血管病。除贫血和血小板减少外，神经功能障碍在TTP中很常见。一名42岁女性因血小板减少导致严重阴道出血入住妇产科。输注血小板后，患者出现意识水平下降、意识模糊、头痛和发热。CT扫描未显示病理变化。经颅多普勒超声显示所有基底脑动脉血流速度增加。由于怀疑为脑炎，患者被转至神经科。脑脊液和脑磁共振成像检查均正常。最后，外周血涂片发现裂红细胞以及乳酸脱氢酶显著升高，从而诊断为TTP。连续3天进行血浆置换后，患者完全缓解。实验室检查（ADAMTS13活性<5%，抗ADAMTS13 IgG抗体）确诊了该诊断。输注血小板可能会对疑似TTP患者的预后产生不利影响。血管性血友病因子裂解蛋白酶（ADAMTS13）严重缺乏活性是血栓性血小板减少性紫癜的特异性表现。
