Módolo Norma Sueli P, de Azevedo Vera Lucia Fernandes, Santos Paulo Sérgio S, Rosa Márcia Leal, Corvino Dina Rita, Alves Lucas Jorge S Castro
Universidade Estadual Paulista Júlio de Mesquita Filho.
Rev Bras Anestesiol. 2010 Mar-Apr;60(2):176-80, 102-4. doi: 10.1016/s0034-7094(10)70023-4.
Factor XI deficiency is a rare hematologic disorder. Hemophilia C (factor XI deficiency) affects both genders and it is usually asymptomatic, manifesting only as postoperative hemorrhage. It is an autosomal recessive, homozygous or heterozygous, disorder, and its severity depends on the levels of factor XI. The objective of this report was to present the anesthetic strategy in a patient with hemophilia C.
This is a 32 years old female, gravida 1/para 0, on the 39th week of pregnancy, scheduled for elective cesarean section. Physical and laboratorial exams did not show any abnormalities. According to the recommendations of the hematologist, on the day of the procedure, the patient was given promethazine, 25 mg, hydrocortisone, 500 mg, due to prior transfusion reaction, and plasma, 10 mL x kg(-1) for a total of 700 mL. Two hours later, the patient underwent subarachnoid block under routine monitoring. Ringer's lactate, 2000 mL, was administered for hydration. The anesthetic-surgical procedure proceeded without intercurrences. Postoperatively, the patient was doing well when, on the 3rd PO day, fresh frozen plasma (FFP), 10 mL x kg(-1), was administered to prevent late postoperative bleeding.
The objective of this report was to present the anesthetic protocol for patients with hemophilia C and to alert for the need of investigation in patients with a history of postoperative bleeding, when a coagulation study should e be done before any invasive procedure and, in the case of prolonged aPTT, one should investigate the presence of factor XI deficiency.
因子XI缺乏症是一种罕见的血液系统疾病。血友病C(因子XI缺乏症)男女均可发病,通常无症状,仅在术后出血时才表现出来。它是一种常染色体隐性、纯合或杂合性疾病,其严重程度取决于因子XI的水平。本报告的目的是介绍一名血友病C患者的麻醉策略。
这是一名32岁女性,孕1产0,妊娠39周,计划行择期剖宫产术。体格检查和实验室检查均未发现任何异常。根据血液科医生的建议,在手术当天,由于既往输血反应,给予患者异丙嗪25mg、氢化可的松500mg,并输注血浆10mL/kg,共700mL。两小时后,患者在常规监测下接受蛛网膜下腔阻滞。输注乳酸林格氏液2000mL进行补液。麻醉-手术过程顺利,无并发症。术后,患者情况良好,术后第3天口服时,给予新鲜冰冻血浆(FFP)10mL/kg,以预防术后晚期出血。
本报告的目的是介绍血友病C患者的麻醉方案,并提醒对于有术后出血史的患者需要进行检查,在任何侵入性操作前应进行凝血研究,若活化部分凝血活酶时间(aPTT)延长,应检查是否存在因子XI缺乏症。