Demirci Ufuk, Erbilen Esra Altan, Ümit Elif Gülsüm, İnan Cihan, Sayın N Cenk, Demir Ahmet Muzaffer
Department of Hematology, Trakya University, Medical Faculty, Edirne, Turkey.
Department of Obstetrics and Gynecology, Trakya University, Medical Faculty, Edirne, Turkey.
Obstet Med. 2023 Sep;16(3):203-205. doi: 10.1177/1753495X211067119. Epub 2022 Jan 13.
Bernard Soulier Syndrome (BSS) is an inherited bleeding disorder characterized by macrothrombocytopenia and absence of ristocetin-induced platelet aggregation. Clinical findings vary from person to person. Most of the patients are diagnosed with muco-cutaneous bleeding such as purpura, epistaxis and gingival bleeding in early childhood. Few pregnant women with BSS are described in the literature. Management of thrombocytopenia during pregnancy and delivery requires a multidisciplinary approach. The family should be warned about the potentially life-threatening bleeding during pregnancy and the delivery and the decision about mode of delivery should be individualised, involving discussion with patient and multidisciplinary team.
伯纳德-索利尔综合征(BSS)是一种遗传性出血性疾病,其特征为巨血小板减少症以及瑞斯托霉素诱导的血小板聚集缺乏。临床症状因人而异。大多数患者在幼儿期被诊断为有黏膜皮肤出血,如紫癜、鼻出血和牙龈出血。文献中描述的患有BSS的孕妇很少。孕期和分娩期间血小板减少症的管理需要多学科方法。应告知家属孕期和分娩期间可能危及生命的出血情况,分娩方式的决定应个体化,需与患者及多学科团队进行讨论。