Davoodabadi Abdolhossein, Adib Mohammad Mahdi, Keleidari Behrooz
Department of General and Thoracic Surgery, Kashan University of Medical Sciences, Kashan, Iran.
Iran J Med Sci. 2011 Jun;36(2):136-40.
Hemophilia A is a bleeding disorder caused by defective production of factor VIII. The main concern associated with the disease is bleeding, especially after trauma and surgeries. Factor VIII replacement therapy is associated with substantial decrease of bleeding events during surgery. However, there have been a number of reports of thromboemblic events in this situation. The present report describes a case of moderate hemophilia A in which splenectomy did lead to pulmonary embolism and subsequent death.The patient was a 25-year-old man with hemophilia A admitted after a car accident and trauma to left lower chest and abdomen. He received factor VIII concentrates for replacement therapy. He was hemodynamically stable on the first day, but on the second day his hemoglobin declined and he showed signs of abdominal tenderness. He, therefore, was subjected to laparatomy and splenectomy. After the operation, he suddenly developed dyspnea and decline in blood pressure, and death afterwards. Autopsy of the patient revealed massive pulmonary thromboembolism. The symptoms and outcome of the present case indicate that although pulmonary thromboembolism in the early postoperative period in patients with hemophilia A undergoing splenectomy and receiving factor VIII concentrate for replacement is rare, it should not be assumed a far-fetched event, and prophylactic measures to prevent thromboemboly must be considered.
甲型血友病是一种由于凝血因子 VIII 生成缺陷引起的出血性疾病。与该疾病相关的主要问题是出血，尤其是在创伤和手术后。凝血因子 VIII 替代疗法可显著减少手术期间的出血事件。然而，在这种情况下已有许多血栓栓塞事件的报告。本报告描述了一例中度甲型血友病患者，其脾切除术后确实发生了肺栓塞并随后死亡。该患者是一名 25 岁的甲型血友病男性，在遭遇车祸并左下胸部和腹部受伤后入院。他接受了凝血因子 VIII 浓缩物进行替代治疗。第一天他血流动力学稳定，但第二天血红蛋白下降，且出现腹部压痛迹象。因此，他接受了剖腹手术和脾切除术。术后，他突然出现呼吸困难和血压下降，随后死亡。对该患者的尸检显示有大量肺血栓栓塞。本病例的症状和结果表明，尽管接受凝血因子 VIII 浓缩物替代治疗的甲型血友病患者在脾切除术后早期发生肺血栓栓塞的情况罕见，但不应将其视为牵强附会的事件，必须考虑采取预防血栓栓塞的措施。
