Wang S X, Guan Y, Nie Y B, Li H Y, Sun B Y, Wang X Y, Yang R C
Institute of Hematology & Blood Disease Hospital, CAMS & PUMC, Tianjin 300020, China.
Zhonghua Xue Ye Xue Za Zhi. 2017 May 14;38(5):404-409. doi: 10.3760/cma.j.issn.0253-2727.2017.05.010.
To analyze the difference of bleeding frequency, plain radiographic (X-ray) , risk factors in hemophilic arthropathy progression and the Arnold-Hilgartner classification. 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. 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) 13 (1-40) ; annualized joint bleeding rate 13.5 (0-38) 8 (0-33) , especially in moderate hemophilia [26 (1-48) 12 (1-36) , <0.001; 18 (0-36) 7.5 (0-26) , =0.001], but severe hemophilia had no difference in bleeding frequency [33 (1-41) 26 (1-40) , =0.702; 22 (0-36) 18 (0-33) , =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 (=0.063, =0.004) . However, similar associations were not found in HB patients (=0.045, =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 (=1.941, =0.052) . However, significant difference between moderate HA and HB was observed (=0.076, =0.002) . Multivariate unconditioned Logistic analysis showed that annualized joint bleeding rate [<0.001, =1.166 (95% 1.097-1.239) ] and articular structural injuries [0.018, =2.842 (95% 1.196-6.755) ] were independent risk factors for the joints radiographic progression. 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.
分析血友病性关节病进展过程中的出血频率、X线平片表现、危险因素及Arnold-Hilgartner分级的差异。对2007年1月至2010年12月在我院医疗中心住院的211例血友病患者进行回顾性研究，部分关节积血患者随访5年。所有患者均为男性，其中甲型血友病（HA）150例，乙型血友病（HB）61例。HA患者出血频率高于HB患者，年化总出血率分别为20.5（0 - 48）和13（1 - 40）；年化关节出血率分别为13.5（0 - 38）和8（0 - 33），尤其是在中度血友病患者中[分别为26（1 - 48）和12（1 - 36），<0.001；18（0 - 36）和7.5（0 - 26），=0.001]，但重度血友病患者在出血频率上无差异[分别为33（1 - 41）和26（1 - 40），=0.702；22（0 - 36）和18（0 - 33），=0.429]。对108例HA患者和54例HB患者的患关节情况进行X线评估。在HA患者中，Arnold-Hilgartner分级随严重程度评分增加而升高（=0.063，=0.004）。然而，在HB患者中未发现类似关联（=0.045，=0.082）。5年后，36例HA患者和19例HB患者接受相同关节X线检查，HA组和HB组总体关节X线进展无显著差异（=1.941，=0.052）。然而，中度HA和HB之间观察到显著差异（=0.076，=0.002）。多因素非条件Logistic分析显示，年化关节出血率[<0.001，=1.166（95% 1.097 - 1.239）]和关节结构损伤[0.018，=2.842（95% 1.196 - 6.755）]是关节X线进展的独立危险因素。该研究表明，HA和HB在出血表型上存在差异，尤其是在中度血友病患者中。与HA患者相比，HB患者随时间推移表现为轻度但渐进性发展。年化关节出血率和关节结构损伤是关节X线进展的独立危险因素。
