Acquired hemophilia B is a rare bleeding disorder in which circulating specific antibodies inhibit the coagulation factors. Usually, it is associated with autoimmune diseases, malignancy and some infections such as hepatitis B (HBV) and C (HCV) viruses. The association with the human immunodeficiency virus (HIV) is rare. We present the first case of hemophilia B acquired in a patient with HIV. A 36-year-old woman with a history of HIV infection stage 3 acquired immunodeficiency syndrome (AIDS) (CDC 2014) and glomerulopathy secondary to HIV. She consulted for subacute non-dysenteric diarrhea. During her hospitalization, bacteremia by methicillin-sensitive Staphylococcus aureus (S.aureus) was documented, requiring hemodialysis catheter replacement with profuse bleeding during the procedure. The coagulation tests showed prolonged activated partial thromboplastin time (aPTT), normal prothrombin time (PT), mixing test with Rosner index: 8.8%, negative lupus anticoagulant, and specific tests for normal factor VIII and XI. The activity of factor IX was <50% and the inhibitor was increased, which confirms the diagnosis of hemophilia B. Acquired hemophilia B in a patient with HIV infection is not a frequent association; it should be suspected in the context of bleeding with prolonged aPTT.

To investigate the feature of underlying disorders, clinical symptoms, diagnosis and treatment strategies of patients with acquired hemophilia(AH). The clinical data and laboratory tests results of 22 patients with AH from March 2010 to June 2014 were retrospectively analyzed. A total of 22 patients with AH were enrolled in our study, including 20 patients diagnosed as acquired hemophilia A (AHA) and 2 as acquired hemophilia B (AHB). Among the AHA patients, there were 10 males and 10 females with the median age of 37.5 (range, 2-95) years old. The median activity of FVIII (FVIII:C) of the 20 AHA patients was 1.9% (0.5%-39.0%). Soft tissue hematoma (80.0%) and subcutaneous bleeding (75.0%) were the most common clinical symptoms. Two male children were diagnosed as AHB (age 1 and 3 years old, respectively) with mild bleeding symptoms, and the activities of FIX (FIX:C) were 5.0% and 16.0%, respectively. In addition, an underlying disorder was found in 7 patients (31.8%). In laboratory testing, all patients had prolonged APTT, normal PT, decreased FVIII:C or FIX:C, positive antibody screening test or antibody titer (2-32 BU), and negative for lupus anticoagulant and anticardiolipin antibody. Nineteen out of 20 patients were treated with blood products to stop acute bleeding episodes. Corticosteroid alone was applied to 7 patients, corticosteroid combined with other immunosuppressive agents to 11 patients, rituximab to 3 patients. Nineteen patients responded well to hemostatic treatment, except 1 patient who died of fatal bleeding. The FVIII:C of 8 patients increased to a normal level with the median time of 42.5(21-145) days. After treatment, the activity of FIX:C of the 2 AHB patients achieved 35% and 24% in 48 and 60 days, respectively. Acquired hemophilia is not an uncommon disease in clinical practices, which can occur in people of all ages. AH is a bleeding disorder with heterogeneous characteristics. Compared with adult, the clinical symptoms of children patients were mild, which lead to underdiagnosis. 探讨获得性血友病的病因、临床表现、临床诊断与治疗。 对2010年3月至2014年6月诊断的22例获得性血友病患者的临床资料进行回顾性分析。 22例患者中，获得性血友病A(AHA)20例(90.9%)，获得性血友病B(AHB)2例(9.1%)。AHA患者中，男、女各10例，中位年龄37.5 (2~95)岁，中位凝血因子Ⅷ活性(FⅧ∶C)为1.9%(0.5%~39.0%)，以肌肉软组织血肿(80.0%)、皮肤瘀斑(75.0%)为主要临床表现；2例AHB患者均为男性儿童（1、3岁各1例），临床症状轻微，凝血因子Ⅸ活性(FⅨ∶C)分别为5.0%、16.0%。22例患者中，7例(31.8%)存在相关病因。所有患者均APTT延长，PT正常，APTT纠正试验不能纠正或检出抑制物（滴度值2~32 BU），狼疮抗凝物及抗心磷脂抗体阴性。19例患者接受血制品止血治疗，7例患者单独使用肾上腺糖皮质激素清除抑制物，11例患者接受肾上腺糖皮质激素联合其他免疫抑制剂清除抑制物，3例患者接受利妥昔单抗治疗。20例AHA患者中19例(95.0%)急性出血控制，8例(40.0%)患者抑制物消失且FⅧ∶C>50%[中位治疗时间42.5(21~145)d]，因出血死亡1例。2例AHB患者分别在治疗48、60 d后FⅨ∶C达到35%、24%。 获得性血友病并非罕见，可见于各年龄段，临床表现异质性高，儿童患者症状较轻。