Zanon E, Zerbinati P, Girolami B, Bertomoro A, Girolami A
Institute of Medical Semeiotics, University of Padua Medical School, Italy.
Blood Coagul Fibrinolysis. 1999 Apr;10(3):117-20. doi: 10.1097/00001721-199904000-00001.
The development of inhibitor antibodies is one of the more important complications in the management of haemophilia patients. In a previous study, the prevalence of inhibitor varies between 5 and 52%, seems to be different for different types of concentrates, and is less frequent in multitransfused patients. In our prospective study we followed for 3 years 62 multitransfused haemophilia patients without inhibitor or past history of inhibitor. Thirty-seven haemophilia patients were treated with intermediate purity factor VIII concentrates, whereas 25 were given high purity concentrates (from the eighth month of the study five of these patients were treated with recombinant products). Factor VIII inhibitor antibody developed in seven of 25 haemophilia patients treated with high purity concentrates or recombinant products, whereas none of the haemophilia patients treated with intermediate purity concentrates had inhibitors. The difference was statistically significant (P < 0.001; OR = 0.06, 95% CI 0.001-0.3). In all patients, the titre of the inhibitor was low and no problem occurred in their management. Since inhibitors appeared in multitransfused patients when transfused with high purity concentrates and/or when the patients were switched to recombinant FVIII product, the development of inhibitor seems to be due to the administration of a new concentrate. Therefore this potential complication must be considered every time a new concentrate is administered.
抑制性抗体的产生是血友病患者治疗中较为重要的并发症之一。在先前的一项研究中，抑制物的发生率在5%至52%之间，似乎因不同类型的浓缩物而异，且在多次输血的患者中发生率较低。在我们的前瞻性研究中，我们对62例无抑制物或无抑制物既往史的多次输血的血友病患者进行了3年的随访。37例血友病患者接受中等纯度的凝血因子VIII浓缩物治疗，而25例患者接受高纯度浓缩物治疗（从研究的第八个月起，其中5例患者接受重组产品治疗）。在接受高纯度浓缩物或重组产品治疗的25例血友病患者中，有7例产生了凝血因子VIII抑制性抗体，而接受中等纯度浓缩物治疗的血友病患者均未产生抑制物。差异具有统计学意义（P < 0.001；OR = 0.06，95% CI 0.001 - 0.3）。在所有患者中，抑制物的滴度较低，其治疗过程中未出现问题。由于在多次输血的患者输注高纯度浓缩物时和/或患者改用重组FVIII产品时出现了抑制物，抑制物的产生似乎是由于使用了新的浓缩物。因此，每次使用新的浓缩物时都必须考虑这种潜在的并发症。
