Creutzig U, Hoelzer D
Klin Padiatr. 1987 May-Jun;199(3):169-72. doi: 10.1055/s-2008-1026784.
Myelodysplastic syndromes (MDS) are heterogeneous diseases with cytopenia in the peripheral blood, dysplasia of two or three cell lines, and a low leukemic blast count in the bone marrow and peripheral blood. The syndrome is rare in childhood (1%-2% of all acute leukemias). In the advanced subtypes of MDS with 5% to 30% of blasts the risk of progression into acute leukemia is high, especially in childhood. Cytogenetic abnormalities like monosomy 7 are typical for children with MDS, and probably are an unfavourable prognostic factor. The overall prognosis is almost the same as in adults, with a median survival time of 19 months. There are no well-established therapy strategies in MDS. Intensive chemotherapy may be beneficial, at least in children and young adults, but the decision when to start treatment is difficult. At present allogeneic bone marrow transplantation is the only curative therapy available for MDS.
骨髓增生异常综合征（MDS）是一类异质性疾病，外周血出现血细胞减少，两系或三系发育异常，骨髓和外周血中白血病原始细胞计数低。该综合征在儿童期罕见（占所有急性白血病的1%-2%）。在骨髓原始细胞占5%至30%的MDS晚期亚型中，进展为急性白血病的风险很高，尤其是在儿童期。像7号染色体单体这样的细胞遗传学异常是MDS患儿的典型特征，可能是一个不良预后因素。总体预后与成人几乎相同，中位生存时间为19个月。MDS尚无成熟的治疗策略。强化化疗可能有益，至少对儿童和年轻成人有益，但决定何时开始治疗很困难。目前，异基因骨髓移植是MDS唯一可用的治愈性疗法。
