血友病A和血友病B患者出血和关节病表现差异的回顾性分析

OBJECTIVE: To analyze the difference of bleeding frequency, plain radiographic (X-ray), risk factors in hemophilic arthropathy progression and the Arnold-Hilgartner classification. METHODS: A retrospective study was conducted in 211 hemophilia patients hospitalized in our medical center between January 2007 and December 2010, some patients with hemarthrosis were followed up for 5 years. RESULTS: All patients were male, including 150 hemophilia A (HA) and 61 hemophilia B (HB). The HA patients bled more frequently than HB patients with annualized total bleeding rate 20.5 (0–48) vs 13 (1–40); annualized joint bleeding rate 13.5 (0–38) vs 8 (0–33), especially in moderate hemophilia [26 (1–48) vs 12 (1–36), P<0.001; 18 (0–36) vs 7.5 (0–26), P=0.001], but severe hemophilia had no difference in bleeding frequency [33 (1–41) vs 26 (1–40), P=0.702; 22 (0–36) vs 18 (0–33), P=0.429]. The condition of the affected joints of 108 HA and 54 HB was evaluated on roentgenography. In HA patients, the Arnold-Hilgartner classification increased with the severity ratings (r=0.063, P=0.004). However, similar associations were not found in HB patients (r=0.045, P=0.082). Five years later, 36 HA and 19 HB patients received the same joint X-ray, there were no significant differences in joints radiographic progression between the total HA and HB groups (z=1.941, P=0.052). However, significant difference between moderate HA and HB was observed (z=0.076, P=0.002). Multivariate unconditioned Logistic analysis showed that annualized joint bleeding rate [P<0.001, OR=1.166 (95%CI 1.097–1.239)] and articular structural injuries [P=0.018, OR=2.842 (95% CI 1.196–6.755)] were independent risk factors for the joints radiographic progression. CONCLUSION: The study suggests that there was a difference in bleeding phenotype between HA and HB, especially in moderate hemophilia. HB patients showed mild but progressive development over time, compared with HA patients. Annualized joint bleeding rate and articular structural injuries were independent risk factors for the joints radiographic progression.