摘要目的 分析血友病A(HA)和血友病B(HB)的出血频率和X线分级情况、X线分级进展的危险因素及差异.方法 回顾性分析2007年1月至2010年12月中国医学科学院血液病医院收治的211例血友病患者的临床资料,随访部分患者出血关节的X线Amold-Hilgartner诊断分级结果.结果 211例患者均为男性,其中HA 150例,HB 61例.HA组总体中位年均总出血次数、中位年均关节出血次数分别为20.5(0～48)、13.5(0～38)次,HB组总体中位年均总出血次数、中位年均关节出血次数分别为13(1～40)、8(0～33)次,中间型HA患者中位年均总出血次数和中位年均关节出血次数高于中间型HB患者[26(1～48)次对12(1～36)次,P＜0.001;18 (0～36)次对7.5(0～26)次,P=0.001],而重型HA和HB患者中位年均总出血次数和中位年均关节出血次数比较差异无统计学意义[33(1～41)次对26(1～40)次,P=0.702;22 (0～36)次对18(0～33)次,P=0.429].108例HA、54例HB患者入院时行出血关节普通X线检查,HA患者血友病严重程度与关节X线Amold-Hilgartner诊断分级呈正相关(r=0.063,P=0.004);HB患者严重程度分级与关节X线Amold-Hilgartner诊断分级无相关性(r=0.045,P=0.082);36例HA和19例HB患者5年后复查病变关节X线片,总体HA与HB患者X线进展程度比较差异无统计学意义(z=1.941,P=0.052),但中间型HA患者进展程度高于中间型HB患者(z=0.076,P=0.002),重型HA和重型HB患者比较差异无统计学意义(z=3.139,P=0.945).多因素分析显示关节出血次数[P＜0.001,OR=1.166(95％CI 1.097～1.239)]和外伤[P=0.0 18,OR=2.842 (95％CI 1.196～6.755)]是血友病患者关节X线Amold-Hilgartner诊断分级进展的独立危险因素.结论 HB患者出血频率低于HA患者,中间型患者差异尤其明显.中间型HB患者关节X线分级进展较中间型HA患者慢.关节出血次数和外伤是血友病患者关节X线Arnold-Hilgartner诊断分级进展的独立危险因素.

abstractsObjective 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),P9=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.