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Postpartum thrombotic microangiopathic syndrome

发布于：2013年4月15日    文字：【大】【中】【小】

Transfus Apher Sci. 2013 Feb;48(1):51-7.

Postpartum thrombotic microangiopathic syndrome.

Owens MY, Martin JN Jr, Wallace K, Keiser SD, Parrish MR, Tam Tam KB, Martin RW.

Abstract

OBJECTIVE: Characterization of syndromes for patients with life-threatening, progressively worsening hemolysis-elevated-liver-enzymes-and-platelet (HELLP) syndrome-like diseases and with thrombotic microangiopathies. RETROSPECTIVE STUDY DESIGN: Patients who underwent postpartum plasma-exchange (PPEX) for preeclampsia-related, and microangiopathy/coagulopathy illnesses unresponsive to medical therapy between 1994 and 2008 in our center and elsewhere.RESULTS: Nine patients were treated with PPEX in our center with 78% maternal survival. Treatment with PPEX increased platelet levels (p=0.048), decreased serum lactic dehydrogenase (p=0.0012) and aspartate aminotransferase (p=0.0001).CONCLUSION: Nineteen patients from publications combined with our patients suggest five categories of postpartum thrombotic microangiopathy syndrome that exhibit HELLP syndrome criteria and respond to PPEX

摘要：

该文章提出产后血栓性微血管病综合征（postpartum thrombotic microangipathic syndrome, PTMS，或者HELLPsyndrome-like clinical and laboratory picture）概念，表现为产后7天以内出现HELLP综合征样临床、实验室表现，伴或不伴多器官功能损害，病情持续进展，传统药物和/或大剂量糖皮质激素治疗无效，其可以包括先兆子痫、HELLP综合征、TTP/HUS、败血症。产后HELLP综合征/先兆子痫和TTP/HUS的发病机制及预后差别很大，但是其鉴别非常困难；HELLP综合征也可以诱发本来存在基因易感性的患者出现TTP；帮助鉴别诊断的实验室检查（如ADAMTS 13活性）需要花费时间；血浆置换延迟将严重影响TTP/HUS的预后；因此，对于疾病初期不能明确诊断的产后TMA患者（ 即PTMS），应尽早开始血浆置换，其中包括败血症和胎盘早剥引起的PTMS（但需个体化）。基于上述理论，本报道总结了9例符合PTMS诊断，但不符合TTP、HUS和ITP诊断标准，并行血浆置换的患者，存活率78%，血浆置换使血小板上升、LDH和AST下降。结论，传统药物及糖皮质激素治疗无效、恶化的PTMS患者应尽早开始血浆置换。

http://www.ncbi.nlm.nih.gov/pubmed/22704633