[一例老年患者的获得性因子VIII血友病]。

Walter Boddi, Stefania Machetti, Nicola Sodi, Lucia Sammicheli, Graziella Cati, Giovanna Marotta, Laura Sabatini, Roberto Cappelli
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引用次数: 0

摘要

获得性血友病是一种罕见的成人凝血病,与出血并发症有关。虽然这种疾病的病因尚不清楚,但一种自身免疫机制产生了抗因子VIII的自身抗体。大约一半的病例与其他疾病有关,主要是产后、潜在癌症、自身免疫性疾病。一名81岁男性因大面积血肿(颈部、胸部、手臂和下肢)入院。没有家族或个人先天性出血史。他患有慢性支气管炎和脑血管疾病;在出现上述症状前一个月内未使用任何药物。实验室参数显示:血红蛋白10.9 g%,血小板计数和白细胞正常,活化部分凝血活酶时间延长(98 s),凝血酶原时间和纤维蛋白原浓度正常。一项活化的部分凝血活酶时间混合研究没有显示任何校正,提示是一种凝血抑制剂。狼疮抗凝血和抗心磷脂抗体均为阴性。生化、免疫及肿瘤标志物检查均正常。胸部和腹部计算机断层扫描未显示病理图像或血肿。凝血因子分析显示凝血因子VIII降低(< 2%)和凝血因子VIII抑制剂升高(55 Bethesda单位)。诊断为特发性获得性血友病。开始输注红细胞和人因子VIII (2000 U/天,连用7天),并静脉滴注甲基强的松龙。观察到临床条件和实验室参数的逐步改善。18天后,患者出院并给予强的松治疗。随访时临床情况和实验室参数均正常。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Acquired factor VIII hemophilia in a geriatric patient].

Acquired hemophilia is a rare coagulopathy in adults, associated with bleeding complications. Although the etiology of this disorder remains obscure, an autoimmune mechanism produces the development of autoantibodies against factor VIII. About half of cases are associated with other conditions, mainly post-partum, underlying cancer, autoimmune disease. An 81-year-old male was admitted to the hospital with extensive hematomas (neck, chest, arms and lower limbs). There was no family or personal history of congenital bleeding diathesis. He had chronic bronchitis and cerebrovascular disease; no drugs had been used during the month prior to noted symptoms. Laboratory parameters revealed: hemoglobin 10.9 g%, normal platelet count and white blood cells, prolonged activated partial thromboplastin time (98 s), with normal prothrombin time and fibrinogen concentration. An activated partial thromboplastin time mixing study did not show any correction, suggesting a coagulation inhibitor. Lupus anticoagulant and anticardiolipin antibodies were negative. Biochemical, immunological tests and tumor markers were normal. Thoracic and abdominal computed tomographic scan did not reveal pathological images or hematomas. Analysis of clotting factors revealed decreased factor VIII (< 2%) and elevated factor VIII inhibitor (55 Bethesda units). Idiopathic acquired hemophilia diagnosis was made. Red blood cell transfusion and human factor VIII (2000 U/day for 7 days) infusion were initiated, intravenously with methylprednisolone. A progressive improvement in clinical conditions and laboratory parameters was observed. After 18 days the patient was discharged and treated with prednisone. At follow-up control the clinical conditions and laboratory parameters were normal.

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