Delbrück C, Haferland I, Scholz K, Asmelash G, Makki V, Müller S, Krekeler S, Alesci S, Miesbach W
Institute of Transfusion Medicine, Goethe University Hospital, Frankfurt, Germany.
Hamostaseologie. 2011 Nov;31 Suppl 1:S24-8.
The retrospective cohort study surveys the influence of age, co-morbidity and laboratory values on FVIII-activity (FVIII:C) in patients with haemophilia A with (mild n = 48, moderate n = 10, severe n = 7 and carriers n = 23). Median observation was 19 years for patients with haemophilia A and 9,5 years for carriers.
FVIII:C levels collected from patients with mild haemophilia A displayed a significant median increase of 6.5% with proceeding age (p = 0.0013). Patients with moderate haemophilia A (and carriers of haemophilia A) showed a non significant median increase of 1.05% (carriers 8%). Eight patients showed FVIII:C levels at last blood withdrawal that indicated a change of severity from moderate to mild haemophilia A. A significant correlation was found between FVIII:C and VWF:RCo (p = 0.0203) and AFP (p < 0.0005). The correlation between FVIII:C and triglycerides and LDH was significant negative (p < 0.0005). No significant correlation could be found for FVIII:C and co-morbidity, fibrinogen, cholesterol and VWF:Ag.
这项回顾性队列研究调查了年龄、合并症和实验室检查值对甲型血友病患者（轻度48例、中度10例、重度7例及携带者23例）FVIII活性（FVIII:C）的影响。甲型血友病患者的中位观察时间为19年，携带者为9.5年。
轻度甲型血友病患者的FVIII:C水平随年龄增长中位数显著增加6.5%（p = 0.0013）。中度甲型血友病患者（及甲型血友病携带者）中位数增加1.05%（携带者为8%），无显著差异。8例患者最后一次采血时的FVIII:C水平表明其严重程度从中度甲型血友病转变为轻度。FVIII:C与VWF:RCo（p = 0.0203）和AFP（p < 0.0005）之间存在显著相关性。FVIII:C与甘油三酯和LDH之间的相关性为显著负相关（p < 0.0005）。未发现FVIII:C与合并症、纤维蛋白原、胆固醇和VWF:Ag之间存在显著相关性。
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