血细胞减少患者衰变加速因子和膜反应性溶血抑制物表达缺失检测的临床意义

目的检测血细胞减少患者外周血红细胞和中性粒细胞细胞膜糖基磷脂酰肌醇(GPI)连接的补体调节蛋白衰变加速因子(CD55)和膜反应性溶血抑制物(CD59)表达情况,并探讨其临床意义。方法 2006年7月-2011年3月,采用直接免疫荧光标记法流式细胞仪检测182例血细胞减少患者外周血CD55及CD59表达情况,其中阵发性睡眠性血红蛋白尿(PNH)9例,再生障碍性贫血(AA)-PNH综合征8例,AA 83例,骨髓增生异常综合征51例,自身免疫性溶血性贫血11例,造血功能停滞6例,缺铁性贫血7例,巨幼细胞性贫血4例,脾功能亢进3例。结果 PNH及AA-PNH患者CD55、CD59抗原缺失率均较其他血细胞减少者明显增高。结论流式细胞仪检测外周血中红细胞和中性粒细胞膜CD55和CD59抗原表达缺失率是目前诊断PNH可靠和敏感的方法,也是对PNH、AA-PNH早期诊断敏感指标,并且PNH克隆检测还能为诊断疾病提供鉴别诊断依据。

Clinical Significance of the Detection of CD55,CD59 Expression Deletion in Patients with Cytopenia

Objective To explore the expression of decaying accelerating factor(CD55) and membrane inhibitor of reactive lysis(CD59) connected with glycosylphosphatidylinositol(GPI) on the membranes of peripheral blood cells and neutrophils in patients with cytopenia,and research on their clinical significance.Methods From July 2006 to March 2011,By directing immunofluorescence flow cytometry,we detected the expression levels of CD55 and CD59 in 182 patients with cytopenia.Among them,there were 9 cases of paroxysmal nocturnal hemoglobinuria(PNH),8 cases of aplastic anemia-PNH(AA-PNH),83 cases of aplastic anemia(AA),51 cases of myelodysplastic syndrome,11 cases of autoimmune hemolytic anemia,6 cases of aplastic crisis,7 cases of iron deficiency anemia,4 cases of megaloblastic anemia and 3 cases of hypersplenism.Results CD55 and CD59 antigens deficiency was more significant in PNH,AA-PNH patients than in other cytopenic patients.Conclusions Detection of CD55 and CD59 antigens deficiency by flow cytometry may act as a sensitive and reliable marker of PNH diagnosis and early diagnosis of PNH and AA-PNH.Moreover,PNH cloning detection can also provide differential diagnosis for cytopenia diseases.