Thomas Mari R, Robinson Susan, Scully Marie A
Department of Haematology, UCLH, Cardiometabolic programme- NIHR UCLH/UCL BRC, London, UK.
Department of Haematology, GSTT, London, UK.
Br J Haematol. 2016 Jun;173(6):821-30. doi: 10.1111/bjh.14045. Epub 2016 Mar 27.
Differentiation between the thrombotic microangiopathies (TMAs) that present in pregnancy may be clinically challenging, but is critical to ensure correct management because of the impact on fetal and maternal outcomes. Thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) are medical/obstetric emergencies that require specialist input, both at the time of acute diagnosis and follow-up in subsequent pregnancies. Features of preeclampsia and HELLP syndrome (haemolysis, elevated liver enzymes, low platelets) may precede or be present in evolving TTP or aHUS. Clinicians need to be mindful of how a presumed diagnosis of a specific TMA in pregnancy may evolve and be prepared to frequently reassess signs and symptoms and revise the diagnosis and management plan accordingly.
妊娠期出现的血栓性微血管病（TMA）之间的鉴别在临床上可能具有挑战性，但由于其对胎儿和母亲结局的影响，对于确保正确管理至关重要。血栓性血小板减少性紫癜（TTP）和非典型溶血性尿毒症综合征（aHUS）是医疗/产科急症，在急性诊断时以及后续妊娠的随访中都需要专科医生的参与。子痫前期和HELLP综合征（溶血、肝酶升高、血小板减少）的特征可能在进展性TTP或aHUS之前出现或与之并存。临床医生需要留意妊娠期特定TMA的假定诊断可能如何演变，并随时准备频繁重新评估体征和症状，并相应地修订诊断和管理计划。
