Zaidi Syed Mohammad Asad, Qureshi Rahat Najam, Adil Salman Naseem
Aga Khan University, Medical College, Karachi, Pakistan.
J Pak Med Assoc. 2010 Feb;60(2):136-8.
Factor VII deficiency is one of the 'rare inherited disorders of coagulation.' Few cases of Factor VII deficiency have been reported during pregnancy, a state which could potentially cause fatal haemorrhage. Here we report a case of a pregnant lady with a history of heavy menorrhagia and multiple first pregnancy failures. Delivery was carried out via Caesarean section due to non-reassuring foetal heart monitoring. Patient was treated with Fresh Frozen Plasma (FFPs) and Factor VII concentrates, however, the patient developed bleeding postoperatively. Literature indicates that whilst Factor VII levels rise during pregnancy in normal women, no increase is seen in homozygous cases, whereas there is a moderate rise in heterozygous individuals. History of heavy menorrhagia, multiple first pregnancy failures and a positive family history for bleeding disorders necessitate investigation and monitoring of Factor VII levels during pregnancy. Factor VII concentrates achieve adequate homeostasis in most cases. Recombinant Factor VIIa, however, is the treatment of choice and does not carry a risk of infection transmission or thrombus formation.
凝血因子 VII 缺乏症是“罕见的遗传性凝血障碍”之一。孕期报道的凝血因子 VII 缺乏症病例很少,而这种情况可能会导致致命性出血。在此,我们报告一例有月经过多病史且多次首次妊娠失败的孕妇病例。由于胎儿心率监测结果不佳,通过剖宫产进行分娩。患者接受了新鲜冰冻血浆(FFP)和凝血因子 VII 浓缩剂治疗,然而,患者术后出现了出血情况。文献表明,正常女性孕期凝血因子 VII 水平会升高,而纯合子病例中未见升高,杂合子个体则有中度升高。月经过多病史、多次首次妊娠失败以及出血性疾病家族史阳性,使得孕期有必要对凝血因子 VII 水平进行检测和监测。大多数情况下,凝血因子 VII 浓缩剂可实现足够的内稳态。然而,重组凝血因子 VIIa 是首选治疗方法,且不存在感染传播或血栓形成的风险。