Jackson N, Hashim Z A, Zainal N A, Jamaluddin N
Department of Medicine, Hospital Universiti Sains Malaysia, Kelantan.
Singapore Med J. 1995 Apr;36(2):230-1.
A 30-year-old Malay lady, with no previous or family history of bleeding, presented with severe gum bleeding 25 days post-partum. The factor VIII:c was 0.03 iu/ml with evidence of a slow-acting factor VIII inhibitor. Von Willebrand factor antigen (VWF:age) varied from less that 0.05 to 0.17 iu/ml, and there was absent ristocetin-induced platelet aggregation. Anti-nuclear and anti-DNA antibodies were present, but there were no other features of systemic lupus erythematosus. There was some clinical response to cryoprecipitate and tranexamic acid, and slight improvement with corticosteroid. Fifteen months later, the patient has no active bleeding problem, and her VWF-ag is increasing spontaneously. However, factor VIII:c is less than 0.01 iu/ml and her factor VIII inhibitor titre is still > 20 Bethesda units/ml.
一名30岁的马来女性，既往及家族均无出血史，产后25天出现严重牙龈出血。因子VIII:c为0.03 iu/ml，有慢效因子VIII抑制剂的证据。血管性血友病因子抗原（VWF:Ag）在0.05至0.17 iu/ml之间变化，瑞斯托霉素诱导的血小板聚集缺失。存在抗核抗体和抗DNA抗体，但无系统性红斑狼疮的其他特征。对冷沉淀和氨甲环酸有一定临床反应，使用皮质类固醇后有轻微改善。15个月后，患者无活动性出血问题，其VWF:Ag正在自发升高。然而，因子VIII:c小于0.01 iu/ml，其因子VIII抑制剂滴度仍>20贝塞斯达单位/ml。
