本文报道了1例以神经功能受损为主诉的血栓性血小板减少性紫癜患者的诊治过程。该患者男性65岁,急性起病,症状表现为失语、肢体麻木及无力,症状波动而多变,并伴有发热。血液学检查发现贫血、血小板计数下降;有轻度肾功能受损;外周血涂片见红细胞碎片;血浆ADAMTS13活性显著降低,ADAMTS13抑制物阳性。该例患者被诊断为特发性血栓性血小板减少性紫癜,经及时诊断及积极进行血浆置换治疗后,病情得以缓解。 This article reports the diagnosis and treatment of a thrombotic thrombocytopenic purpura, a predominant complaint of neurological impairment. The patient, male, 65 years old, had an acute onset of symptoms characterized by aphasia, numbness and weakness in his limbs, varied and fluctuating symptoms, accompanied by fever. Hematology examination revealed anemia, decreased platelet count; mild renal impairment; peripheral blood smear see red blood cell debris; plasma ADAMTS13 activity was significantly reduced, ADAMTS13 inhibitor positive. The patient was diagnosed with idiopathic thrombotic thrombocytopenic purpura, after timely diagnosis and active plasma exchange therapy, the disease was alleviated.