Rehberg Jonathan F, Briery Christian M, Hudson William T, Bofill James A, Martin James N
Department of Obstetrics and Gynecology, University of Mississippi Medical Center, Jackson, MS 39216-4505, USA.
Obstet Gynecol. 2006 Sep;108(3 Pt 2):817-20. doi: 10.1097/01.AOG.0000215994.25958.31.
Thrombotic thrombocytopenic purpura rarely presents during late pregnancy or immediately postpartum. This report describes the clinical course of a patient considered to have hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome but later determined to have thrombotic thrombocytopenic purpura.
At 37 weeks of gestation, a multiparous woman was diagnosed with HELLP syndrome. She received high-dose dexamethasone, magnesium, antihypertensives, and platelets before delivery. Over the next 36 hours, renal function acutely worsened and death ensued. One week after death a plasma ADAMTS13 activity of 4% was reported.
Thrombotic thrombocytopenic purpura can mimic HELLP syndrome late in gestation. Lack of response to dexamethasone within 12-24 hours and atypical relationships among laboratory values are two clues that thrombotic thrombocytopenic purpura may be the underlying pathology and that plasma exchange is emergently needed.
血栓性血小板减少性紫癜在妊娠晚期或产后即刻很少出现。本报告描述了一名患者的临床病程，该患者最初被认为患有溶血、肝酶升高、血小板减少（HELLP）综合征，但后来被确定为血栓性血小板减少性紫癜。
一名经产妇在妊娠37周时被诊断为HELLP综合征。她在分娩前接受了大剂量地塞米松、镁剂、抗高血压药物和血小板治疗。在接下来的36小时内，肾功能急剧恶化并导致死亡。死亡一周后报告血浆ADAMTS13活性为4%。
血栓性血小板减少性紫癜在妊娠晚期可酷似HELLP综合征。12 - 24小时内对地塞米松无反应以及实验室检查值之间的非典型关系是两个线索，提示血栓性血小板减少性紫癜可能是潜在病因，急需进行血浆置换。
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