Fujiwara F, Kanoh G, Ogawa H, Takaya K, Hibi S, Shima M, Yoshioka A, Imashuku S
Department of Pediatrics, Nantan General Hospital.
Rinsho Ketsueki. 1997 Dec;38(12):1263-8.
This report describes the transient development of inhibitors in two cases of hemophilia A. Case 1 was an 8-month-old boy who suffered from subcutaneous hematoma with extremely low level (1.5%) of plasma factor VIII activity (F VIII:C). He showed an inhibitor titer of 2.5 Bethesda units (BU)/ml when he suffered from measles at age 24 months with poor clinical response to factor VIII. This inhibitor elevated to 11.6 BU/ml at 29 months, but completely disappeared at 32 months. Case 2 was a 5-month-old boy who suffered from subcutaneous hematoma with extremely low level (< 1%) of plasma F VIII:C. At 8 months his inhibitor titer peaked at 19 BU/ml but subsequently declined to 11.5 BU/ml at 12 months and completely disappeared at 14 months. A review of the literature revealed that the median (range) titer of transient inhibitor initially developed at 2 (0.5-19) BU/ml on 225 (79-448) days, immediately peaked at 3.6 (0.5-60) BU/ml and disappeared by 665 (282-1140) days, suggesting that careful monitoring of F VIII inhibitors is required in the treatment of hemophilia A.
本报告描述了两例甲型血友病患者体内抑制剂的短暂发展情况。病例1是一名8个月大的男孩，患有皮下血肿，血浆凝血因子VIII活性（F VIII:C）极低（1.5%）。他在24个月大患麻疹时出现抑制剂滴度为2.5贝塞斯达单位（BU）/毫升，对凝血因子VIII的临床反应不佳。该抑制剂在29个月时升至11.6 BU/毫升，但在32个月时完全消失。病例2是一名5个月大的男孩，患有皮下血肿，血浆F VIII:C水平极低（<1%）。在8个月时，他的抑制剂滴度峰值为19 BU/毫升，但随后在12个月时降至11.5 BU/毫升，并在14个月时完全消失。文献回顾显示，短暂性抑制剂的滴度中位数（范围）最初在225（79 - 448）天出现时为2（0.5 - 19）BU/毫升，随即在3.6（0.5 - 60）BU/毫升达到峰值，并在665（282 - 1140）天内消失，这表明在甲型血友病的治疗中需要仔细监测F VIII抑制剂。
