Angchaisuksiri P, Atichartakarn V, Pathepchotiwong K, Jootar S, Ungkanont A, Chuncharunee S
Division of Hematology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Southeast Asian J Trop Med Public Health. 1993;24 Suppl 1:152-8.
Eleven cases of acquired inhibitors against factor VIII: C and von Willebrand's factor (vWF) seen at the Department of Medicine, Ramathibodi Hospital from 1979 to 1991 were reviewed. Factor VIII: C inhibitor was found in 6 of 36 patients (17%) with hemophilia A (median age 18 years). Three patients each were weak (titer < 10 Bethesda units/ml), and strong antibody producers. Two cases of weak antibody producers had spontaneous disappearance of inhibitor, while all 3 strong antibody producers required specific treatment (corticosteroids, immunosuppressive drugs, and plasmapheresis). The inhibitor level temporarily declined in 2 patients, and disappeared in one. Spontaneous acquired inhibitor to factor VIII: C was seen in 3 patients. One each respectively had pemphigus vulgaris and bullous pemphigoid, autoimmune disease, and NIDDM. They were characterized by older age (median age 54 years), frequent skin and soft-tissue hematoma, but less hemarthroses. Inhibitor titer ranged from 15-280 Bethesda units/ml. Disappearance of the inhibitor after treatment with corticosteroids and immunosuppressive drugs were observed in all patients. Acquired von Willebrand's disease developed in 2 previously healthy patients. One patient was in the postpartum period, while the other had simultaneous acute viral hepatitis A infection. Both presented with the recent onset of spontaneous severe gingival bleeding, and demonstrated a prolonged bleeding time, reduced vWF:Ag (F VIIIR:Ag), and ristocetin cofactor (F VIIIR:vWF). Treatment with cryoprecipitate and corticosteroid resulted in remission of bleeding symptoms. Despite the rarity of these disorders, the recognition and proper management are of importance.
对1979年至1991年间在拉玛蒂博迪医院内科所见的11例获得性抗凝血因子VIII：C和血管性血友病因子（vWF）抑制剂病例进行了回顾。在36例甲型血友病患者（中位年龄18岁）中，发现6例存在因子VIII：C抑制剂（17%）。3例患者产生的是弱抑制剂（滴度<10贝塞斯达单位/毫升），另外3例是强抗体产生者。2例弱抗体产生者的抑制剂自发消失，而所有3例强抗体产生者都需要进行特殊治疗（使用皮质类固醇、免疫抑制药物和血浆置换）。2例患者的抑制剂水平暂时下降，1例患者的抑制剂消失。3例患者出现了自发获得性因子VIII：C抑制剂。分别有1例患有寻常型天疱疮、大疱性类天疱疮、自身免疫性疾病和非胰岛素依赖型糖尿病。他们的特点是年龄较大（中位年龄54岁），频繁出现皮肤和软组织血肿，但关节积血较少。抑制剂滴度范围为15 - 280贝塞斯达单位/毫升。所有患者在使用皮质类固醇和免疫抑制药物治疗后抑制剂均消失。2例既往健康的患者发生了获得性血管性血友病。1例患者处于产后，另1例同时感染了急性甲型病毒性肝炎。两人均近期出现自发性严重牙龈出血，出血时间延长，血管性血友病因子抗原（F VIIIR：Ag）和瑞斯托霉素辅因子（F VIIIR：vWF）降低。使用冷沉淀和皮质类固醇治疗后出血症状缓解。尽管这些疾病罕见，但认识和正确处理很重要。
