Immunosuppressive Treatments for Myelodysplastic Syndromes
Immunosuppressive Treatments for Myelodysplastic Syndromes
复制标题
骨髓增生异常综合征的免疫抑制治疗
作者：
T. Shimamoto;K. Ohyashiki
骨髓增生异常综合征（MDS）包括异质组克隆造血干细胞疾病，而MDS患者经常观察到免疫异常。几份报告显示，约10%的MDS患者有临床自身免疫性疾病，如皮肤血管炎、风湿病或自身免疫性溶血性贫血。此外，在18-65%的MDS患者中发现血清学免疫异常，如高或低γ -球蛋白血症、抗核抗体阳性、直接库姆斯试验阳性或CD4/8比值倒置。最近免疫抑制疗法包括强的松龙、抗胸腺细胞球蛋白和环孢素A （CsA）被用于治疗一些MDS患者的细胞减少症。我们观察了CsA在50例MDS患者中的疗效。30例（60%）患者血液学改善，特别是红系患者。良好核型或DRB1*1501型应答者明显多于中间/差核型或其他HLA型应答者。MDS伴红细胞发育不全是一种罕见的MDS，目前尚未明确定义。我们报告了4例MDS合并红细胞发育不全的患者，他们有骨髓发育异常和红细胞前体百分比低的形态学证据。通过Southern blot和PCR分析，在这些患者中发现了TCR- β和- γ基因的重排。此外，他们使用TCR曲目分析扭曲了TCR的使用。他们的贫血在接受CsA治疗后得到显著改善。我们必须建立免疫抑制治疗在MDS患者中的临床有效性，并建立简单的工具来揭示t细胞介导的骨髓抑制在个体患者中的决策。
The myelodysplastic syndromes (MDS) comprise a heterogeneous group of clonal hematopoietic stem cell disorders, while, immunological abnormalities are frequently observed in patients with MDS. Several reports revealed that about 10% of MDS patients have clinical autoimmune disorders like skin vasculitis, rheumatic disease, or autoimmune hemolytic anemia. Furthermore, serological immunological abnormalities like hyper- or hypogammaglobulinemia, positivities of antinuclear antibody, positivities of direct Coombs test, or inverted CD4/8 ratios were found in 18-65% of patients with MDS. Recently immunosuppressive therapies including prednisolone, antithymocyte globulin, and cyclosporin A (CsA) are used to treat cytopenia in some patients with MDS. We examined the efficacy of CsA in 50 patients with MDS. Hematologic improvement was observed in 30 (60%) patients especially for erythroid lineage. There were significantly more responders with good karyotype or DRB1*1501 than with intermediate/poor karyotypes or with other HLA types. MDS with erythroid hypoplasia is a rare form of MDS, and has not yet been clearly defined. We reported four patients with MDS with erythroid hypoplasia who had morphological evidence of myelodysplasia and low percentage of erythroid precursors. Rearrangements of the TCR- β and - γ genes were seen in these patients using Southern blot and PCR analysis. Also they had skewed TCR usages using TCR repertoire analysis. Their anemia drastically improved with CsA therapy. We have to establish the clinical usefulness of immunosuppressive therapy in MDS patients and simple tools for revealing T-cell mediated myelosuppression in the individual patients for decision-making.
阵发性睡眠性血红蛋白尿症的分子基础。
影响因子：
2.9
作者：
Yomtovian,R;Prince,GM;Medof,ME
通讯作者：
Medof,ME
影响因子：
20.3
作者：
Charles, RJ;Sabo, KM;Abkowitz, JL
通讯作者：
Abkowitz, JL
影响因子：
2.7
作者：
Epperson, DE;Nakamura, R;Barrett, AJ
通讯作者：
Barrett, AJ