Mielot F, Buisine J, Duchayne E, Fenneteau O, Goasguen J, Guitard A M, Maier-Redelsperger M, Malet M, Manel A M
Laboratories of Hematology: Hôpital Bicêtre, Toulouse, France.
Leuk Lymphoma. 1998 Feb;28(5-6):531-40. doi: 10.3109/10428199809058361.
We reviewed the peripheral blood and bone marrow smears of 81 children with myelodysplastic syndrome (MDS). The morphological FAB classification was applicable in 59 children (72.8%): RAEB and RAEBt were the most frequent, 32 cases (39.5%). CMML was observed in 15 cases (18.5%) and in 25% of them, serological evidence for a recent EBV infection was demonstrated. In 22 cases (27.2%), the FAB classification was not convenient. In some of these children, dysmyelopoiesis was associated with constitutional disorders. Among these various inherited conditions, Down syndrome in which myelodysplasia is the expression of an abnormal clonal hematopoiesis, and mitochondrial cytopathies in which MDS is the hematological expression of a polyclonal multi-organ disease. The FAB classification does not appear to be satisfactory for all the disorders included in the group of childhood MDS and should be modified for specific use in children.
我们回顾了81例小儿骨髓增生异常综合征（MDS）患儿的外周血和骨髓涂片。形态学FAB分类适用于59例患儿（72.8%）：难治性贫血伴原始细胞过多（RAEB）和难治性贫血伴原始细胞过多转变型（RAEBt）最为常见，共32例（39.5%）。15例（18.5%）观察到慢性粒-单核细胞白血病（CMML），其中25%的患儿有近期EB病毒感染的血清学证据。22例（27.2%）患儿不适用FAB分类。在其中一些患儿中，骨髓生成异常与先天性疾病有关。在这些各种遗传性疾病中，骨髓发育异常是异常克隆性造血表现的唐氏综合征，以及骨髓增生异常综合征是多克隆多器官疾病血液学表现的线粒体细胞病。FAB分类对于儿童MDS组中所包含的所有疾病似乎并不令人满意，应针对儿童的具体使用进行修改。
