Kesteven P J, Holland L J, Lawrie A S, Savidge G F
Thromb Haemost. 1984 Aug 31;52(1):50-2.
Factor VIII inhibitors in mild haemophilia are uncommon and the management of such patients is controversial. The development of a persistently high responding F VIII inhibitor in a mild haemophiliac is reported and the behaviour of the inhibitor discussed in the context of the various therapeutic regimes employed for symptomatic management. When inhibitor titres were low, endogenous F VIII stimulation, by DDAVP, was less immunogenic than the administration of exogenous F VIII concentrates. This inhibitor displayed characteristics of an autoantibody, and was characterised as an immunoglobulin of IgG subtype.
轻度血友病患者中出现因子 VIII 抑制剂的情况并不常见,对此类患者的治疗存在争议。本文报道了一名轻度血友病患者出现持续高反应性因子 VIII 抑制剂的情况,并在用于症状性治疗的各种治疗方案背景下讨论了该抑制剂的行为。当抑制剂滴度较低时,去氨加压素对内源性因子 VIII 的刺激比给予外源性因子 VIII 浓缩物的免疫原性更低。这种抑制剂表现出自身抗体的特征,并被鉴定为 IgG 亚型的免疫球蛋白。