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一名先天性无纤维蛋白原血症女性的妊娠相关血栓形成:两例成功妊娠的报告

Pregnancy-related thrombosis in a woman with congenital afibrinogenemia: a report of two successful pregnancies.

作者信息

Roqué H, Stephenson C, Lee M J, Funai E F, Popiolek D, Kim E, Hart D

机构信息

Department of Obstetrics and Gynecology, New York University Medical Center, 550 First Avenue, New York, NY 10016, USA.

出版信息

Am J Hematol. 2004 Jul;76(3):267-70. doi: 10.1002/ajh.20110.

Abstract

We managed two pregnancies in a woman with congenital afibrinogenemia with increasing amounts of cryoprecipitate to achieve a pre-infusion fibrinogen level of 60 mg/dL. The first pregnancy resulted in placental abruption at 36 weeks, in spite of recent cryoprecipitate infusion. Both placentas showed infarction. Post-partum ovarian and renal vein thromboses complicated the second pregnancy. Mean FVIII (344%) and vWF Antigen (323%) were elevated prior to cryoprecipitate infusion, with mean post-infusion levels of 367% and 363%. The clearance of fibrinogen after cryoprecipitate infusion increased during the course of pregnancy. A paradoxical prothrombotic state with embolization may play a role in the observed complications of pregnancy.

摘要

我们对一名患有先天性纤维蛋白原血症的女性进行了两次妊娠管理,使用了越来越多的冷沉淀,以使输注前纤维蛋白原水平达到60mg/dL。尽管最近输注了冷沉淀,但第一次妊娠在36周时发生了胎盘早剥。两个胎盘均显示梗死。产后卵巢和肾静脉血栓形成使第二次妊娠复杂化。冷沉淀输注前平均FVIII(344%)和vWF抗原(323%)升高,输注后平均水平分别为367%和363%。冷沉淀输注后纤维蛋白原的清除率在妊娠过程中增加。伴有栓塞的矛盾性血栓形成状态可能在观察到的妊娠并发症中起作用。

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