Ji L, Yang R, Yang D, Chen Z, Xing S, Tian M, Sun Y
Blood Diseases Hospital, CAMS & PUMC, Tianjin, P. R. China.
Haemophilia. 1998 Sep;4(5):721-4. doi: 10.1046/j.1365-2516.1998.00184.x.
Eight nonhaemophilic patients with factor VIII (FVIII) inhibitors were reported. There was no difference in sex distribution. Median age at diagnosis was 62 years (ranging from 14 to 73 years). No associated disorders were revealed and all the patients were presented with severe muscular or arthral bleeding. Inhibitor titre was measured by the Bethesda method, which were 6.4, 126.0, 155.0, 4.8, 56.0, 13.5, 35.0 and 150.0 BU mL-1, respectively, at diagnosis. FVIII:C levels were less than 1 U dL-1 in seven patients and less than 2 U dL-1 in one patient. The median vWF:Ag level was 210% (ranging from 80% to 340%). All the patients had good response to activated prothrombin complex concentrates for acute bleeding episodes and prednisone for inhibitor elimination. Inhibitors completely eliminated in seven patients within a follow-up duration over 1 year, and one patient died of intracranial haemorrhage when her inhibitor titre decreased to 4.5 BU mL-1 and FVIII:C increased to 21 U dL-1.
报告了8例患有因子VIII（FVIII）抑制剂的非血友病患者。性别分布无差异。诊断时的中位年龄为62岁（范围为14至73岁）。未发现相关疾病，所有患者均出现严重的肌肉或关节出血。抑制剂滴度通过贝塞斯达法测量，诊断时分别为6.4、126.0、155.0、4.8、56.0、13.5、35.0和150.0 BU mL-1。7例患者的FVIII:C水平低于1 U dL-1，1例患者低于2 U dL-1。vWF:Ag水平的中位数为210%（范围为80%至340%）。所有患者对急性出血发作时的活化凝血酶原复合物浓缩物和消除抑制剂时的泼尼松均有良好反应。7例患者在超过1年的随访期内抑制剂完全消除，1例患者在其抑制剂滴度降至4.5 BU mL-1且FVIII:C升至21 U dL-1时死于颅内出血。
