CD5 及其在自身免疫性疾病中的作用研究进展

CD5是主要分布在T淋巴细胞及少量B淋巴细胞表面的大分子糖蛋白,不仅参与固有免疫反应,还调控T、B淋巴细胞介导的特异性免疫反应,并通过TCR和BCR信号通路影响T、B淋巴细胞的生物学特性及功能。CD5在T细胞中通过与CK2结合共同调节后续信号通路的功能及活性,而B细胞中的B1a细胞也可特异性表达CD5使其在免疫过程中发挥作用。自身免疫性疾病是机体对自身抗原发生免疫反应而导致自身组织损害而引起的疾病,研究发现CD5参与多种自身免疫性疾病过程。本文综述CD5及其在自身免疫性疾病中的作用的研究进展,为CD5在自身免疫性疾病的临床靶向治疗提供新观点和研究思路。     

骨髓增生异常综合征造血细胞凋亡的研究进展

骨髓增生异常综合征（ＭＤＳ）是一组恶性克隆性造血干细胞疾病,以血细胞的质、量异常,骨髓病态造血及高风险发展为急性白血病为主要特征。大部分ＭＤＳ患者在髓内造血活跃的同时,却伴有外周血细胞减少。这一矛盾现象被称为ＭＤＳ骨髓和“无效造血”。不少研究表明,过...     

骨髓增生异常综合征染色体异常与白血病发展

目的：探讨在骨髓增生异常基础上白血病发展。方法：骨髓细胞Brdu-SCD细胞周期检测法和R-带染色体核型分析法,在1989年-1998年间对361例MDS(按FAB分类)、11例回顾性诊断白血病前期(PL)和73例MDS-RCMD(按WHO分类)以及140例AA和PNH骨髓细胞进行分析。并对18例MDS-RCMD进行V-erbB基因诊断。还对30例MDS和AA进行动态观察。与此同时,有25例MDS转变为急性白血病,它们几乎都是骨髓细胞SCD阴性患者,与回顾性诊断白前的结果相同。结果：1)361例MDS中MDS-RA占91.3％,核型异常检出率为66.2％,SCD阴性病例占50．9％,与以往报道的结果相似。染色体异常检出率高于文献报道。MDS中两项均不正常的病例占31．5％。2)从和PNH核型异常检出率为13．5％,SCD阴性病例占19．2％。两项指标均明显低于MDS,且两项均不正常患者仅为1．4％。3)MDS,RCMD,AA和回顾性白前有相同染色体异常,但有不同的克隆性异常检出率。4)MDS从SCD阳性转变为阴性,从保持阳性或阴性转变为阳性。结论：1)白前和MDS发展为白血病,或白前转变为MDS而后发展为白血病的共同机制在于骨髓细胞SCD阴性。它可能与胸苷酸代谢异常和胸苷酸合成酶基因扩增并长期积累有关。2)白前、MDS和AA可能有共同的细胞克隆起源。这一点已为AA／AML家系研究所证实。     

多指标综合诊断骨髓增生异常综合征

骨髓增生异常综合征(MDS)是一类造血干细胞恶性克隆性疾病,MDS的诊断历来是国内外血液学家关注的焦点。从1982年FAB分型到2008年WHO分型的修订,MDS的诊断经历了由单纯形态学指标至多指标综合诊断的转变。如今,联合细胞形态学、组织化学、分子遗传学、免疫表型等多指标综合诊断是MDS诊断的主导思想。     

免疫性血小板减少症相关免疫负调控因子研究进展

正ITP患者血小板减少的机制包括血小板消耗过多及产生不足,两者皆主要由IgG抗血小板抗体介导~([1]),自身免疫的主要靶点是血小板糖蛋白,如GPⅡb/Ⅲa和GPⅠb/Ⅸ。已经证实,ITP与血小板自身抗原的失耐受有关,而失耐受的原因仍不清楚。大量的研究表明ITP患者体内也存在着细胞免疫紊     

骨髓单个核细胞Coombs试验阳性的血细胞减少患者补体水平变化及其意义

Objective To investigate the variation of bone marrow complement level in cytopenia pa-tients with positive BMMNC-Coombs test(CBCPC), and probe the role of complement in destroying hemato-poietic cells of CBCPC patients. Methods One hundred and twenty-four patients with CBCPC and twenty-three healthy donors as controls were enrolled in this study. The levels of CI-150, C3, C4, C5b-9 were tested with ELISA. The auto-antibodies on bone marrow hematupoietic cells (BMHC) were examined with flow cy-tometry. Results The level of C5b-9 in bone marrow(BM) of untreated CBCPC patients [(119.8 ± 54.0)μg,/L] was significantly higher than that of recovered patients [(100.7 ± 33.4) μg/L] or normal controls [(93.9 ± 28.8) μg/I.] (P < 0.05). The levels of CH50 in BM of untreated or recovered CBCPC patients [(33.3 ± 11.5) kU/L, (30.8 ± 10.3) kU/L] were significantly higher than that of normal controls [(24.1 ±6.4) kU/L] (P < 0.05). The level of C3 in BM of untreated or recovered CBCPC patients [(4.9 ± 2.2) mg/ L], (5.0 ± 3.5) mg/L] was significantly lower than that of normal controls [(7.0 ± 5.6) mg/L] (P < 0.05). The level of complement in peripheral blood was consistent with that in BM. CH50 in BM of CBCPC patients was negatively correlated with their C3 (r = - 0. 303, P = 0. 007) and positively correlated with their C5b-9(r = 0. 241, P = 0. 003) levels. The level of C5h-9 in BM of CBCPC patients was higher in the BMHC-IgM positive group [(117.6 ± 55.7) μg/L] than in the BMHC- IgM negative group [(99.2 ± 26.2)μg/L] (P < 0. 05). The positive rate of CD34+ -IgG or CD34 + -IgM of CBCPC patients was positively corre-lated with their C5 b-9 level (r = 0. 593, P = 0.000, r = 0. 326, P = 0. 049). The reticulocyte percentage (r =0. 421, P = 0.000) and serum indirect bilirubin level (r = 0. 230, P = 0. 032) of CBCPC patients were posi-tively correlated with their CHSO level. Conclusions The hematocytopenia of CBCPC patients might be re-lated to the hematopoietic cells destruction caused by auto-antibedy activated complements.     

蒽环类药物治疗非霍奇金淋巴瘤心脏毒性的临床研究

蒽环类化疗药物作为一种疗效肯定的抗恶性肿瘤药物在临床上已得到广泛应用,但该类药物的心脏毒性也日益引起人们的关注.现对我院收治的94例非霍奇金淋巴瘤(NHL)患者应用蒽环类化疗药物后出现的心脏毒性进行临床分析.     

骨髓单个核细胞Coombs试验(+)血细胞减少症患者有核红细胞膜EPO受体与自身抗体关系的初步研究

骨髓单个核细胞(BMMNC)Coombs试验(+)的免疫相关性血细胞减少症(IRP)是由于B淋巴细胞分泌多种针对骨髓造血细胞自身抗体引起的外周血细胞减少[1-2].目前推测自身抗体的作用机制之一可能是封闭骨髓造血细胞膜功能抗原进而抑制造血.