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骨髓发育异常综合征(MDS)主要见于成人,儿童罕见。本文按 FAB 协作组提出的诊断标准,报道3例 MDS 患儿,内2例为土耳其血统。例1 诊断为 RAEB-T,在光镜下其骨髓原始细胞形态未能详加识别而明确诊断,然而通过超微结构的研究,证实这些细胞为粒-单核细胞。例2除骨髓前体细胞出现 Auer 小体外,符合 RAEB-T 的诊断。例3与 CMML 较为一致,其单核细胞绝对值未超过1000/mm~3,故而不典型,易误诊,但末梢血单核细胞占有核细胞总数的25%以上。与成人 MDS 相同,3例都有染色体异常,即三体-8、单体-7和 Y 染色体缺失。3例中至少伴有两个造血细胞系异常的表现,均按急非淋诱导治疗后,2例发展为典型的白血
Myelodysplastic syndromes (MDS) are mainly found in adults and children are rare. According to the diagnostic criteria proposed by the FAB collaboration group, 3 cases of MDS were reported, and 2 were of Turkish ancestry. In case 1, RAEB-T was diagnosed as RAEB-T. The morphology of bone marrow blasts was not well defined by light microscopy. However, ultrastructural studies confirmed that these cells were granulocyte-mononuclear cells. Example 2 The diagnosis of RAEB-T is consistent with the appearance of small Auer bodies in bone marrow progenitor cells. Example 3 is more consistent with the CMML, the absolute value of mononuclear cells does not exceed 1000 / mm ~ 3, it is not typical, easily misdiagnosed, but peripheral blood mononuclear cells account for more than 25% of the total number of nuclear cells. As with adult MDS, all three had chromosomal abnormalities, namely trisomy 8, and deletion of monomeric-7 and Y chromosomes. 3 cases with at least two abnormal hematopoietic cell line performance, were treated with acute and non-lymph node therapy, 2 cases developed into a typical white blood