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血栓性血小板减少性紫癜与重度子痫前期:妊娠期的临床重叠及可能的共存。

Thrombotic thrombocytopenic purpura and severe preeclampsia: a clinical overlap during pregnancy and a possible coexistence.

机构信息

Obstetrics and Fetal medicine Unit, CHRU of Nancy, Nancy, France; Department of fetopathology and placental pathology, CHRU of Nancy, F-54000 Nancy, France; Université de Lorraine, Inserm, IADI, F-54000 Nancy, France.

Obstetrics and Fetal medicine Unit, CHRU of Nancy, Nancy, France.

出版信息

J Gynecol Obstet Hum Reprod. 2022 Sep;51(7):102422. doi: 10.1016/j.jogoh.2022.102422. Epub 2022 Jun 7.

DOI:10.1016/j.jogoh.2022.102422
PMID:35688323
Abstract

In pregnant women, Thrombotic Thrombocytopenic Purpura (TTP) mimics severe preeclampsia because of the overlapping of these symptoms. We report the case of a 28-years-old woman who presented severe thrombocytopenia (platelets at 34 G/L) at 31 gestational weeks. The day after, she showed anaemia, worsening thrombocytopenia (platelets at 6 G/L) and an isolated increase of AST (91 UI/L). Plasmapheresis was performed without improvement. Secondary, she presented hypertension and proteinuria at 15 g/24 hours and elevated liver enzymes (AST 116 UI/L). A cesarean section was performed on day four for suspected HELLP syndrome. We observed in postpartum a progressive increase of platelets and regression of hepatic cytolysis. The sFlt1/PlGF ratio was elevated at 855, the results of ADAMTS13 activity were below 5 %, and the ADAMTS13 gene was mutated. TTP and HELLP syndrome can co-exist and can complicate one another. TTP lead to placental malperfusion. Further studies are needed to confirm the diagnosis value of the sFlt-1/PlGF ratio in TTP pregnant women.

摘要

在孕妇中,血栓性血小板减少性紫癜(TTP)由于这些症状重叠,类似于严重的子痫前期。我们报告了一例 28 岁的女性患者,她在 31 孕周时出现严重的血小板减少症(血小板计数为 34G/L)。第二天,她出现贫血、血小板减少加重(血小板计数为 6G/L)和 AST 单独升高(91UI/L)。进行血浆置换后没有改善。其次,她在 15 克/24 小时出现高血压和蛋白尿,且肝酶升高(AST116UI/L)。第四天,由于疑似 HELLP 综合征,进行了剖宫产。我们观察到产后血小板逐渐增加,肝细胞溶解消退。sFlt1/PlGF 比值升高至 855,ADAMTS13 活性结果低于 5%,ADAMTS13 基因发生突变。TTP 和 HELLP 综合征可以同时存在并相互复杂化。TTP 导致胎盘灌注不良。需要进一步的研究来确认 sFlt-1/PlGF 比值在 TTP 孕妇中的诊断价值。

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