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[May-Heggelin异常与妊娠。一例报告及文献复习]

[May-Heggelin anomaly and pregnancy. A case report, review of the literature].

作者信息

Tordjeman N, Metzger N, Valat A S, Dufour P, Goudemand J, Puech F

机构信息

Service de Gynécologie-Obstétrique, Hôpital Jeanne-de-Flandre, CHRU, Lille.

出版信息

J Gynecol Obstet Biol Reprod (Paris). 1996;25(8):836-40.

PMID:9026514
Abstract

The May-Hegglin anomaly is a rare autosomal dominant platelet disorder characterized by thrombocytopenia, giant platelets and existence of crescent-shaped inclusions within the cytoplasm of granulocytes, eosinophils and monocytes (Döhle body). We report a case of May-Hegglin anomaly associated with a pregnancy. The pregnancy and delivery were uneventful. The child is not a carrier of this hematologic anomaly. Nine cases of complicated pregnancies with this anomaly have been reported in the literature. The risks of maternal hemorrhagic accident during pregnancy and during delivery are weak due to the fact that platelets functions are preserved. The same applies to the fetus. Nevertheless, as in the case of maternal autoimmune thrombocytopenic purpura, most reports advice performing a fetal platelet count on fetal blood sampling before birth to decide upon the mode of delivery. The risk of the cordonal approach to perform fetal blood sampling must be balanced against the small fetal hemorrhagic risk and most authors propose to allow normal delivery whilst avoiding all traumatic instrumental extraction, especially the use of vacuum extractor.

摘要

May-Hegglin异常是一种罕见的常染色体显性血小板疾病,其特征为血小板减少、巨大血小板,以及在粒细胞、嗜酸性粒细胞和单核细胞的细胞质内存在新月形包涵体(Döhle小体)。我们报告一例与妊娠相关的May-Hegglin异常病例。妊娠和分娩过程均顺利。该患儿不是这种血液学异常的携带者。文献中已报道9例伴有这种异常的复杂妊娠病例。由于血小板功能得以保留,孕期和分娩期间发生母体出血事故的风险较低。胎儿情况也是如此。然而,如同母体自身免疫性血小板减少性紫癜的情况一样,大多数报告建议在出生前对胎儿进行采血以检测胎儿血小板计数,从而决定分娩方式。进行胎儿采血的脐带穿刺方法的风险必须与较小的胎儿出血风险相权衡,大多数作者建议允许正常分娩,同时避免所有创伤性器械助产,尤其是避免使用真空吸引器。

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