Department of Neurology, Medical University of Silesia Hospital No. 7, Professor Leszek Giec Upper Silesian Medical Centre, Katowice, Poland.
Department of Neurology, Medical University of Silesia Hospital No. 7, Professor Leszek Giec Upper Silesian Medical Centre, Katowice, Poland.
Neurol Neurochir Pol. 2014;48(4):296-8. doi: 10.1016/j.pjnns.2014.07.002. Epub 2014 Jul 11.
Thrombotic thrombocytopenic purpura (TTP, Moschcowitz disease) is characterized by thrombotic microangiopathy leading to microvascular occlusion and ischemic dysfunction of various organs including the brain. In the course of the rare disease most patients develop neurological symptoms of varying severity and characteristics. The case presented is that of a 34-year-old female patient with profound thrombocytopenia, anemia and rapidly progressive neurological deterioration into coma with normal result of brain imaging. TTP was recognized on the basis of hematological analysis. The initiated steroid therapy and plasma exchange failed to prevent the turbulent course of disease in the patient, who died exhibiting symptoms of multiple organ failure caused by thrombotic microangiopathy. TTP remains to be a diagnostic challenge, particularly in the case of atypical symptoms or when neuroimaging and laboratory results are inconclusive. Before using the corticosteroids and plasma exchange, TTP had a case fatality rate of approx. 90% (Podolak-Dawidziak, 2013). Nowadays recovery is possible when vigorous treatment is introduced early in the course of this disease.
血栓性血小板减少性紫癜（TTP，Moschcowitz 病）的特征为血栓性微血管病导致微血管阻塞和包括大脑在内的各种器官的缺血性功能障碍。在这种罕见疾病的病程中，大多数患者会出现不同严重程度和特征的神经症状。本病例报告的是一名 34 岁女性患者，其存在严重血小板减少、贫血和迅速进展为昏迷的神经恶化，而脑部影像学检查结果正常。根据血液学分析诊断为 TTP。尽管启动了类固醇治疗和血浆置换，但仍未能阻止患者病情的急剧恶化，该患者最终死于由血栓性微血管病引起的多器官衰竭症状。TTP 仍然是一个诊断挑战，尤其是在出现非典型症状或神经影像学和实验室结果不确定时。在使用皮质类固醇和血浆置换之前，TTP 的病死率约为 90%（Podolak-Dawidziak，2013）。如今，当在疾病早期积极治疗时，患者有可能康复。
