Kaiser C, Flachowsky S, Riehn A, Distler W
Klinik und Poliklinik für Frauenheilkunde und Geburtshilfe, Universitätsklinik Carl Gustav Carus, Technische Universität Dresden.
Geburtshilfe Frauenheilkd. 1996 Feb;56(2):101-4. doi: 10.1055/s-2007-1022251.
This case report describes for the first time a manifestation of thrombotic thrombocytopenic purpura (TTP, Moschcowitz's disease) during pregnancy. The characteristic neurologic symptoms of TTP were missing, while the typical clinical signs of the HELLP syndrome could be observed. Furthermore the biochemical proof of TTP was obtained by tracing ultra-large clotting factor VIII (von Willebrand factor) multimers in the patient's plasma. Thus the assumption is supported that TTP and HELLP syndrome are diseases of one pathophysiological entity with different symptomatologies. Because of the frequent relapse of TTP, differentiation between TTP and HELLP syndrome is of great clinical importance, independent of future pregnancies.
本病例报告首次描述了妊娠期血栓性血小板减少性紫癜（TTP，Moschcowitz病）的一种表现形式。TTP典型的神经系统症状未出现，而HELLP综合征的典型临床体征却可观察到。此外，通过追踪患者血浆中超大的凝血因子VIII（血管性血友病因子）多聚体，获得了TTP的生化证据。因此，支持了TTP和HELLP综合征是具有不同症状的同一病理生理实体疾病这一假设。由于TTP频繁复发，TTP与HELLP综合征的鉴别在临床上具有重要意义，与未来是否妊娠无关。
