急性溶血性贫血患儿人类骨髓端粒酶催化亚单位的表达

Expression of Human Telmerase Reverase Transcriptase in Bone Marrow of Children withAcute Hemolytic Anemia

目的探讨急性溶血性贫血患儿人类骨髓端粒酶催化亚单位（hTERT）的表达及其与外周静脉血Hb水平的关系。方法急性溶血性贫血患儿15例。男13例,女2例;年龄5.5-11.0岁。其中葡萄糖-6-磷酸脱氢酶（G-6-PD）缺乏症患儿10例;药物诱导性溶血性贫血患儿5例。选择同期本院血液/肿瘤科病房确诊为上呼吸道感染所致的粒细胞减少症、骨髓检查正常患儿10例为对照组;4株K562细胞为阳性对照。采集G-6-PD缺乏症、药物诱导性溶血性贫血和粒细胞减少症患儿外周静脉血,全自动血液分析仪XE2100检测各组患儿外周静脉血Hb水平,采用Zinkham法检测G-6-PD缺乏症和对照组患儿血G-6-PD活性,分析二组患儿血G-6-PD活性的差别。采集急性溶血性贫血组和对照组患儿胸骨骨髓,采用半定量反转录-聚合酶链反应检测其骨髓和阳性对照K562细胞株的hTERT的相对表达水平,比较急性溶血性贫血组与对照组患儿、阳性对照K562细胞株hTERT相对表达水平的差异。常规检测急性溶血性贫血患儿外周血Hb水平。采用Pearson直线相关分析急性溶血性贫血患儿骨髓hTERT表达水平与外周血Hb水平的关系。结果与对照组比较,G-6-PD缺乏症患儿血G-6-PD活性明显下降（t=8.373P=0）。急性溶血性贫血患儿骨髓hTERT相对表达水平显著高于对照组患儿（t=6.052P=0）,但明显低于K562细胞株（t=9.893P=0）。急性溶血性贫血患儿骨髓hTERT表达水平与其外周血Hb水平呈负相关（r=-0.888P=0）。结论急性溶血性贫血患儿急性溶血时,低水平Hb可在一定范围内上调骨髓hTERT表达水平。

Objective To explore the expression of human telmerase reverase transeriptase （hTERT） in bone marrow of children with acute hemolytic anemia and agranuloeytosis, and the relationship between the expression of hTERT and hemoglobin level. Methods Fifteen patients with acute hemolytic anemia （13 male ,2 female） , 10 of them diagnosed as glucose -6 -phosphate dehydrogenase（ G- 6 -PD） deficiency ,5 diagnosed as drug - induced hemolytic anemia. Ten cases with agranuloeytosis caused by upper respiratory infection and normal bone marrow were selected as control group,4 K562 cell lines as positive control group. All cases of the control group were collected from the blood and tumor department wards of the same hospital during the same period. Peripheral vein blood were obtained for the study in all patients. The concentrations of hemoglobin in peripheral vein blood were measured by XE2100. Zinldaam method was used to quantitate the activity of G - 6 - PD in peripheral vein blood from patients with G - 6 - PD deficiency and agranuloeytosis. The difference between the G - 6 - PD deficiency and agranuloeytosis group was done in the G - 6 - PD activity using the student＇s t - test. Also hTERT expression in bone marrow from children with G - 6 - PD deficiency, drug - induced hemolytic anemia and agranuloeytosis and K562 cell lines were investigated using semi - quantitative reverse transeriptase - polymerase chain reaction analysis. Their expression levels of hTERT between the acute hemolytic anemia and agranuloeytosis group as well as K562 cell lines were compared using the student＇s t - test. The relationship between hTERT and hemoglobin was analyzed by the Pearson test in acute hemolytic anemia group. Results G - 6 - PD activity in fire G - 6 - PD deficiency group showed significantly down - regulated than that of agranuloeytosis group （ t = 8. 373 P = 0）. The hTERT mRNA expression level was signifi~ eantly up - regulated in bone marrow from patients with acute hemolytic anemia group compared with agranuloeytosis group（ t = 6. 052 P = 0） ,but significantly lower than K562 cell line （ t = 9. 893 P = 0 ）. Furthermore, a significantly inverse correlation was found between the expression level of hTERT mRNA in bone marrow and the hemoglobin concentration in peripheral blood in acute hemolytic anemia group （ r = - 0. 888 P = 0）. Conclusion The severe anemia with low hemoglobin concentration might upregnlate hTERT expression of bone marrow in patients with acute hemolytic anemia.