Shen Chian-Yin, Fu Lin-Shien, Lin Heng-Kuei, Han Shao-Min, Wang Jiaan-Der
Division of Pediatric Immunology, Taichung Veterans General Hospital, Taipei, Taiwan.
J Pediatr Hematol Oncol. 2012 Nov;34(8):e344-5. doi: 10.1097/MPH.0b013e3182678ec1.
Acquired hemophilia is rarely observed in a pediatric population. We report a case of a 14-year-old girl presented with ecchymoses and macrohematuria. She developed factor VIII and factor IX inhibitors, and was diagnosed with simultaneous acquired hemophilia and systemic lupus erythematosus (SLE). Recombinant-activated FVII and corticosteroid were prescribed due to macrohematuria-related hypovolemia and anemia, which resolved satisfactorily. This case is a reminder that the rare concurrent presence of factor VIII and factor IX inhibitors could be associated with SLE in a pediatric population. Children with SLE-associated-acquired hemophilia may develop macrohematuria as well.
获得性血友病在儿科人群中很少见。我们报告一例14岁女孩，表现为瘀斑和肉眼血尿。她出现了凝血因子VIII和IX抑制剂，被诊断为同时患有获得性血友病和系统性红斑狼疮（SLE）。由于肉眼血尿相关的血容量不足和贫血，给予了重组活化FVII和皮质类固醇治疗，症状得到了满意缓解。该病例提醒我们，凝血因子VIII和IX抑制剂罕见的同时存在可能与儿科人群中的SLE有关。患有SLE相关获得性血友病的儿童也可能出现肉眼血尿。
