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秦芳梅,叶洁瑜,黄顺桦,李志涛,易正山,郑忠信,刘阳,孙竞.血小板活化功能及血小板微粒对重型血友病A临床异质性的意义[J].陆军军医大学学报(原第三军医大学学报),2015,37(13):1362-1366.
Qin Fangmei,Ye Jieyu,Huang Shunhua,Li Zhitao,Yi Zhengshan,Zheng Zhongxin,Liu Yang,Sun Jing.Significance of platelet activating capacity and platelet-derived microparticles in clinical phenotype heterogeneity of hemophlia A: report of 31 cases[J].J Amry Med Univ (J Third Mil Med Univ),2015,37(13):1362-1366.
秦芳梅,叶洁瑜,黄顺桦,李志涛,易正山,郑忠信,刘阳,孙竞.血小板活化功能及血小板微粒对重型血友病A临床异质性的意义[J].陆军军医大学学报(原第三军医大学学报),2015,37(13):1362-1366. DOI:
Qin Fangmei,Ye Jieyu,Huang Shunhua,Li Zhitao,Yi Zhengshan,Zheng Zhongxin,Liu Yang,Sun Jing.Significance of platelet activating capacity and platelet-derived microparticles in clinical phenotype heterogeneity of hemophlia A: report of 31 cases[J].J Amry Med Univ (J Third Mil Med Univ),2015,37(13):1362-1366. DOI:
目的 应用流式细胞术检测血小板表面特异性抗原和血小板微粒(platelet microparticles,PMPs),并探讨血小板活化程度及PMPs对重型血友病A患者临床表现异质性的意义。 方法 应用特异性荧光单抗CD41a-APC
CD42b-PE
CD61-FITC
CD62P-PE
and CD63-PE及标准荧光校准微珠流式细胞术检测血小板微粒,比较不同临床表型重型血友病A患者(FⅧ∶C2%)外周血血小板CD62P和CD63表达率和PMPs水平
其中轻度表型10例(年出血次数6) ,重度表型21例(年出血次数≥24),正常对照者17例。 结果 重度表型组和轻度表型组CD62P阳性率均值高于正常对照组[(36.2±9.27)% vs(22.06±10.72)%
P=0.001];[(49±11.81)% vs (22.06±10.72)%
P=0.000];轻度表型组CD62P表达阳性率高于重度表型组[(49±11.81)% vs(36.2±9.27)%
P=0.0408)]。重度出血表型组和轻度出血表型组CD63阳性率均值高于正常对照组[(34.82±7.13)% vs (19.08±17.05)%
P=0.04; (51.15±14.30)% vs (19.08±17.05)%
P=0.000];轻度表型组较重度表型组CD63阳性率均值高[(51.15±14.3)% vs (34.82±7.13)%
P=0.004 4]。轻度表型组PMPs高于重度表型组及正常对照组高[(2.06± 1.33)% vs (0.95±0.64)%
P=0.02];[(2.06±1.33)% vs (1.06±1.20)%
P=0.02]。 结论 血小板活化功能及血小板微粒是影响重型血友病A临床表型的因素,轻度表型患者血小板活化功能、血小板微粒水平较重度表型高。
Objective To determine the levels of platelet specific antigens and platelet-derived microparticle (PMPs) by flow cytometry (FCM) in hemophlia A patients
and analyze their clinical significances in the phenotype heterogeneity of the patients with severe hemophlia A. Methods Thirty-one patients with severe hemophlia A (aged from 18 to 33
median 23.5) were enrolled in this study. They were 10 with mild type (annualized bleed rates 6) and 21 with moderate and severe type (annualized bleed rates ≥24). Another 17 healthy male volunteers served as normal controls. Their supernatant plasma samples were measured for baseline PMPs count
with phycoerythrin (PE)-conjugated monoclonal antibodies CD41. Fluorescent microspheres of 1.0 μm and standard fluorescent microbeads (0 to 1.0 μm) in diameter were used to set the microparticle gate
and the antibodies of CD41a-APC
CD42b-PE
CD61-FITC
CD62P-PE
and CD63-PE specific to platelets derived from platelets were employed to analyze the expression of corresponding platelet activating antigens by flow cytometry. Results The expression percentage of CD62P and CD63 was obviously higher in the mild type than the sever type patients (mean±SD: 49±11.81 vs 36.2±9.27
P=0.040 8);(mean±SD: 51.15±14.3 vs 34.82±7.13
P=0.004 4) and a higher mean level of PMPs counts in mild type patients compared to severe type (mean±SD: 2.06±1.33 vs 0.95±0.64
P=0.02). Conclusion Platelet activating capacity and PMPs exert certain effect on clinical phenotypes of hemophlia A. The mild patients have stronger platelet activating capacity and higher PMPs count than the severe ones.
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