Clausen N, Petrini P, Claeyssens-Donadel S, Gouw S C, Liesner R
Department of Pediatrics, University Hospital of Aarhus at Skejby, Aarhus, Denmark.
Haemophilia. 2014 Nov;20(6):747-55. doi: 10.1111/hae.12470. Epub 2014 Jun 3.
The bleeding phenotype has been suggested to differ between haemophilia A and B. More knowledge on the bleeding phenotype at initiation of treatment is important to optimize patient care. The aim of this study was to investigate the severity of the bleeding phenotype and the variation in bleeding in children with severe or moderate haemophilia A and B. Consecutive, previously untreated patients with severe or moderate haemophilia A and B (factor VIII or IX activity <0.01 or 0.01-0.05 IU mL(-1) respectively) born between January 1st 2000 and January 1st 2010 were included. Primary outcome was severity of bleeding tendency. Secondary outcome was variation in bleeding pattern. A total of 582 patients with severe haemophilia A and 76 with severe haemophilia B did not differ in age at first exposure to clotting factor (0.81 vs. 0.88 years, P = 0.20), age at first bleed (0.82 vs. 0.88 years, P = 0.36), and age at first joint bleed (1.18 vs. 1.20 years, P = 0.59). Patients with moderate haemophilia were older compared to patients with severe haemophilia. In patients with moderate haemophilia there were no clear differences between haemophilia A and B. Severity and variation in bleeding phenotype are similar during the early stage of treatment in patients with severe and moderate haemophilia A and B respectively. The findings imply that children with haemophilia B should be observed and treated as vigilantly as those with haemophilia A.
有人提出，甲型血友病和乙型血友病的出血表型有所不同。更多关于治疗开始时出血表型的知识对于优化患者护理很重要。本研究的目的是调查重度或中度甲型和乙型血友病患儿出血表型的严重程度以及出血情况的差异。纳入了2000年1月1日至2010年1月1日出生的连续的、先前未接受治疗的重度或中度甲型和乙型血友病患者（分别为因子VIII或IX活性<0.01或0.01 - 0.05 IU mL⁻¹）。主要结局是出血倾向的严重程度。次要结局是出血模式的差异。共有582例重度甲型血友病患者和76例重度乙型血友病患者在首次接触凝血因子的年龄（0.81岁对0.88岁，P = 0.20）、首次出血的年龄（0.82岁对0.88岁，P = 0.36）以及首次关节出血的年龄（1.18岁对1.20岁，P = 0.59）方面没有差异。中度血友病患者比重度血友病患者年龄更大。在中度血友病患者中，甲型和乙型之间没有明显差异。重度和中度甲型和乙型血友病患者在治疗早期出血表型的严重程度和差异分别相似。这些发现意味着，乙型血友病患儿应与甲型血友病患儿一样受到密切观察和治疗。
