Bossi P, Cabane J, Ninet J, Dhote R, Hanslik T, Chosidow O, Jouan-Flahault C, Horellou M H, Leynadier F, Liozon E, Pouchot J, Robin J P, Sanderson F, Schaeffer A, Sicard D, Staikowsky F, Wechsler B, Zittoun R
Hospital Saint Antoine, Paris, France.
Am J Med. 1998 Nov;105(5):400-8. doi: 10.1016/s0002-9343(98)00289-7.
Acquired hemophilia is a rare disease caused by the development of auto-antibodies against factor VIII.
We studied the characteristics and outcomes of 34 patients (19 women and 15 men) with acquired hemophilia from 1980 to 1997.
The mean age of the patients was 61 years (range, 22-93 years). An underlying disease was observed in 18 (53%) patients: 5 patients had cancer, 4 an autoimmune disorder, 2 a dermatologic disorder, 3 asthma, 3 were postpartum, and 1 had an adverse reaction to ampicillin. Factor VIII level was <5% in 30 (90%) patients; factor VIII antibodies were elevated (>10 Bethesda units) in 23 (69%) patients. Bleeding requiring transfusions was reported in 25 (75%) patients. Human factor VIII was given to 14 patients and porcine factor VIII to 5. Six patients received prothrombin complex concentrates and one desmopressin. Several immunosuppressive treatments were used, mainly corticosteroids, cyclophosphamide, and intravenous immunoglobulin. Bleeding stopped in all but one patient within 2 weeks. Most patients achieved complete remission, although two relapses were observed subsequently.
This large study helps to clarify the presentation and clinical course of acquired hemophilia. Prospective studies are needed to determine the efficacy of treatment.
获得性血友病是一种由针对凝血因子VIII的自身抗体产生所导致的罕见疾病。
我们研究了1980年至1997年间34例获得性血友病患者（19名女性和15名男性）的特征及转归。
患者的平均年龄为61岁（范围22 - 93岁）。18例（53%）患者存在基础疾病：5例患有癌症，4例患有自身免疫性疾病，2例患有皮肤病，3例患有哮喘，3例为产后患者，1例对氨苄西林有不良反应。30例（90%）患者的凝血因子VIII水平<5%；23例（69%）患者的凝血因子VIII抗体升高（>10贝塞斯达单位）。25例（75%）患者报告有需要输血的出血情况。14例患者接受了人凝血因子VIII，5例接受了猪凝血因子VIII。6例患者接受了凝血酶原复合物浓缩剂，1例接受了去氨加压素。使用了多种免疫抑制治疗方法，主要是皮质类固醇、环磷酰胺和静脉注射免疫球蛋白。除1例患者外，所有患者的出血在2周内停止。大多数患者实现了完全缓解，不过随后观察到2例复发。
这项大型研究有助于阐明获得性血友病的临床表现和临床病程。需要进行前瞻性研究以确定治疗的疗效。
