Miyata Akira, Yasuda Yukiko, Fujii Souichiro, Kikuchi Takeshi
Department of Internal Medicine, Chugoku Central Hospital, Mutual Aid Association of Public School Teachers, Fukuyama City.
Rinsho Ketsueki. 2002 Oct;43(10):911-7.
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.
12例依赖输血的骨髓增生异常综合征(MDS)患者接受了免疫抑制治疗;8例使用环孢素A(CyA),3例使用CyA联合抗胸腺细胞球蛋白(ATG),1例仅使用ATG。10例患者联合使用了粒细胞集落刺激因子(G-CSF)。8例患者实现了输血独立,其中4例仅接受CyA治疗,3例接受ATG/CyA治疗,1例接受ATG治疗。9例难治性贫血患者中有8例出现反应,3例难治性贫血伴原始细胞增多患者均无反应,不过大多数情况下恢复并不完全。所有对CyA有反应的患者病程均依赖药物。6例有反应的患者治疗后通过流式细胞术检测了糖基磷脂酰肌醇(GPI)锚定蛋白缺陷粒细胞(CD11b+CD55-CD59-)的存在情况,其中2例检测到。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免疫抑制治疗的良好反应相关。所有有反应的患者均为难治性贫血,根据国际预后评分系统(IPSS)分类为中危-1,且有以下共同情况:无染色体异常、从诊断到治疗的间隔时间短、采用了ATG治疗。