Nielsen Ove Juul, Friis-Hansen Lennart
Klinisk Biokemisk Afdeling KB3011, Rigshospitalet, DK-2100 København Ø, Denmark.
Ugeskr Laeger. 2009 Nov 30;171(49):3614-20.
The thrombotic microangiopathic diseases, which include acquired and congenital TTP and HUS, are most frequently acute disease entities. Untreated, these diseases are associated with a lethal course in many cases. Deficiency of the von Willebrand cleaving enzyme, ADAMTS13, is a decisive pathophysiological defect in most cases of thrombotic microangiopathic diseases. Early recognition and a secure distinction from other conditions that resemble TMA diseases is essential in order to institute adequate treatment and to obtain a favourable outcome in terms of both survival and development of serious sequelae.
血栓性微血管病，包括获得性和先天性血栓性血小板减少性紫癜（TTP）及溶血尿毒综合征（HUS），大多为急性疾病实体。若不治疗，这些疾病在许多情况下会导致致命后果。血管性血友病裂解酶（ADAMTS13）缺乏是大多数血栓性微血管病病例中的决定性病理生理缺陷。为了进行充分治疗并在生存及严重后遗症的发生方面取得良好结果，早期识别并与其他类似血栓性微血管病的病症进行明确区分至关重要。
