骨髓增生异常综合征(MDS)是以骨髓病态造血,致外周血细胞质和量异常的一组血液病。临床表现主要是贫血、感染及自发性出血,本症染色体核型异常的发生率高达40～60％。据报道,先天愚型伴MDS的患儿常有伴发疾病如感染,血型不合所致新生儿溶血症、非血型不合所致高胆红素血症、先天性心脏病、肝炎综合征及肝硬化,与本例相同。本病小儿罕见,下面报道一例。患儿,男,21天,主因全身皮肤黄染20天、黑便16天于1989年6月27日入院。患儿 Myelodysplastic syndrome (MDS) is a group of hematological disorders characterized by abnormal bone marrow cytoplasm and abnormalities in the peripheral blood. The main clinical manifestations of anemia, infection and spontaneous bleeding, the incidence of chromosomal abnormalities in this disease as high as 40 to 60%. It is reported that children with Down’s syndrome MDS often associated with diseases such as infection, blood group incompatibility due to neonatal hemolytic disease, non-blood group incompatibility hyperbilirubinemia, congenital heart disease, hepatitis syndrome and liver Hardened, same as this example. The disease is rare in infants, one case is reported below. Children, male, 21 days, the main body due to yellow dye 20 days, black stool 16 days in June 27, 1989 admission. Children