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Outcome of immunosuppressive therapy for myelodysplastic syndromes: results of 12 cases from a single institution

机译:骨髓增生异常综合症免疫抑制治疗的结果:来自单个机构的12例结果

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Twelve transfusion-dependent patients with myelodysplastic syndrome (MDS) were treated with immunosuppressive therapy; 8 with cyclosporin A (CyA), 3 with CyA and antithymocyte globulin (ATG), one with ATG. G-CSF was combined in 10 patients. Eight patients who consisted of 4 treated with CyA, 3 with ATG/CyA, and one with ATG, achieved transfusion-independence. Responses were observed in 8/9 patients with refractory anemia, 0/3 patients with refractory anemia with excess of blast, although the recovery was incomplete in most cases. All of the CyA-responsive patients took drug-dependent courses. The presence of GPI-anchored protein-deficient granulocytes (CD11b+CD55-CD59-) was examined by flow cytometry after treatment in 6 responsive patients, and was demonstrated in 2 of them. HLA-DR15 was found in 5 of 7 responsive patients, suggesting that the presence of this allele may be associated with a good response to immunosuppressive therapy. All responsive patients had refractory anemia classified to IPSS Int-1, and had common conditions as follows: absence of chromosomal abnormality, short interval from diagnosis to therapy, and employment of ATG therapy.
机译:12例输血依赖性骨髓增生异常综合征(MDS)患者接受了免疫抑制治疗; 8个使用环孢菌素A(CyA),3个使用CyA和抗胸腺细胞球蛋白(ATG),一个使用ATG。 G-CSF合并10例患者。 8例患者,其中4例接受CyA治疗,3例接受ATG / CyA治疗,1例接受ATG治疗,实现了输血独立性。在8/9难治性贫血患者中观察到反应,0/3难治性贫血伴有大量母细胞的患者,尽管大多数情况下恢复并不完全。所有对CyA有反应的患者都接受了药物依赖课程。治疗后通过流式细胞术检查了6例有反应的患者中是否存在GPI锚定的蛋白质缺陷型粒细胞(CD11b + CD55-CD59-),并在其中2例中进行了证实。在7例有反应的患者中有5例发现了HLA-DR15,提示该等位基因的存在可能与对免疫抑制疗法的良好反应有关。所有反应性患者均具有IPSS Int-1分类的难治性贫血,并具有以下常见情况:无染色体异常,从诊断到治疗的间隔时间短以及采用ATG治疗。

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