Sokołowska Bozena, Tomczak Waldemar, Gromek Tomasz, Legieć Wojciech
Klinika Hematoonkologii i Transplantacji Szpiku AM w Lublinie.
Pol Arch Med Wewn. 2006 Apr;115(4):351-5.
We present two cases with a history of acquired haemophilia with massive haemorrhage in the course of the disease. A 74-year-old man presented to the emergency room with an extensive, progressive swelling and haemorrhage in the region of left knee joint, both upper legs and forearms. Laboratory studies revealed the presence of the factor VIII inhibitor in the titer of 115 Bethesda U/mL, low level of factor VIII activity (19.2%) and severe anemia (Hb - 7.0 g%). The patient was treated with FFP transfusions and prednisone with cyclophosphamide to eradicate factor VIII inhibitor. The remission was achieved and lasts for a two years. A 52-year-old woman presented to the emergency room with an extensive subcutaneous haemorrhage in the region of right knee joint and right lower leg. Laboratory studies revealed the presence of the factor VIII inhibitor in the titer of 30 Bethesda U/mL. The factor VIII activity level was only 4%. The patient did not receive the FFP because the severity of the haemorrhage was low. She was treated with steroids. The factor VIII inhibitor disappeared after 2.5 months of therapy.
我们报告两例获得性血友病患者,在疾病过程中出现大量出血。一名74岁男性因左膝关节、双侧大腿和前臂区域广泛、进行性肿胀及出血就诊于急诊室。实验室检查显示,VIII因子抑制物滴度为115 Bethesda单位/毫升,VIII因子活性水平低(19.2%),且有严重贫血(血红蛋白-7.0克%)。该患者接受新鲜冰冻血浆输注及泼尼松联合环磷酰胺治疗以消除VIII因子抑制物。病情缓解并持续了两年。一名52岁女性因右膝关节和右小腿区域广泛皮下出血就诊于急诊室。实验室检查显示,VIII因子抑制物滴度为30 Bethesda单位/毫升。VIII因子活性水平仅为4%。由于出血程度较轻,该患者未接受新鲜冰冻血浆治疗。她接受了类固醇治疗。治疗2.5个月后,VIII因子抑制物消失。