Jedidi Inès, Hdiji Sondes, Ajmi Naourez, Makni Faiza, Masmoudi Sayda, Elloumi Moez, Kallel Choumous
CHU Habib Bourguiba, laboratoire d'hématologie, Sfax.
Ann Biol Clin (Paris). 2011 Nov-Dec;69(6):685-8. doi: 10.1684/abc.2011.0638.
Acquired haemophilia is a rare disease; it occurs most frequently in elderly patients. The majority of cases are due to autoantibodies to factor VIII, which deplete circulating factor VIII or acquired haemophilia A. Only few cases of acquired haemophilia B are reported until today. We report a case of a 7-year-old girl with no past medical history of bleeding disorder and who present an extensive haematoma in the left calf. The diagnosis was established by the demonstration of an isolated prolongation of the activated partial thromboplastin time (APTT) with a reduced factor IX level and evidence of factor IX inhibitor activity to 2 Bethesda Unit (2UB). Diagnosis of acquired haemophilia B confirmed, patient received recombinant factor VIIa and corticosteroid treatment. Bleeding symptoms had completely disappeared and coagulation tests become normal. In conclusion, if bleeding symptoms are associated with unexplained prolongation of APTT, an inhibitor against factor must be searched for not missing an acquired coagulation disease.
获得性血友病是一种罕见疾病;它最常发生于老年患者。大多数病例是由于针对凝血因子VIII的自身抗体,该抗体消耗循环中的凝血因子VIII或导致获得性血友病A。截至目前,仅报道了少数几例获得性血友病B。我们报告一例7岁女童,既往无出血性疾病病史,左小腿出现广泛血肿。通过活化部分凝血活酶时间(APTT)单独延长、凝血因子IX水平降低以及凝血因子IX抑制活性达2贝塞斯达单位(2UB)得以确诊。确诊为获得性血友病B后,患者接受了重组凝血因子VIIa和皮质类固醇治疗。出血症状完全消失,凝血试验恢复正常。总之,如果出血症状与无法解释的APTT延长相关,必须查找针对凝血因子的抑制剂,以免漏诊获得性凝血疾病。