伴有发育异常性骨髓嗜酸性粒细胞增多和inv(16)的急性粒单核细胞白血病之前的骨髓增生异常综合征

Myelodysplastic syndrome preceding acute myelomonocytic leukemia with dysplastic marrow eosinophilia and inv(16).

作者信息

Horiike S, Misawa S, Nishida K, Nishigaki H, Tsuda S, Taniwaki M, Takino T, Abe T

机构信息

Third Department of Internal Medicine, Kyoto Prefectural University of Medicine, Japan.

出版信息

Acta Haematol. 1989;82(3):161-4. doi: 10.1159/000205368.

DOI:10.1159/000205368

PMID:2510440

Abstract

A 44-year-old Japanese male having refractory anemia with excess of blasts (RAEB) preceding acute myelomonocytic leukemia (AMMoL) with dysplastic marrow eosinophilia (M4Eo in the FAB classification) is reported. Sequential cytogenetic studies revealed a specific chromosomal abnormality, inv(16) (p13q22), when RAEB was first diagnosed and again when overt leukemic transformation compatible with M4Eo was manifested. However, during the interval between the RAEB and AMMoL, an unrelated abnormal karyotype without inv(16), 47,XY,+9, was seen, when hematological data revealed remission after a low dose of cytosine arabinoside was administered. In this patient eosinophils in the marrow were involved in the leukemic process cytogenetically, because a few metaphases overlaid with eosinophilic granules had the inv(16) with other numerical abnormalities.

摘要

报告了一名44岁的日本男性，其在急性粒单核细胞白血病（FAB分类中的M4Eo）伴发育异常性骨髓嗜酸性粒细胞增多之前患有难治性贫血伴原始细胞增多（RAEB）。连续的细胞遗传学研究显示，在首次诊断RAEB时以及出现与M4Eo相符的明显白血病转化时，均存在一种特定的染色体异常，即inv(16)(p13q22)。然而，在RAEB和AMMoL之间的间隔期，当血液学数据显示在给予低剂量阿糖胞苷后病情缓解时，发现了一种与inv(16)无关的异常核型，即47,XY,+9。在该患者中，骨髓中的嗜酸性粒细胞在细胞遗传学上参与了白血病过程，因为一些覆盖有嗜酸性颗粒的中期细胞除了有其他数量异常外还存在inv(16)。