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目的报告1例以眼眶绿色瘤起病的伴t(8;21)的儿童M4Eo,并综合国内外文献进行讨论,以提高对该病的认识。方法对此例患儿的临床表现、实验室检查、诊断和治疗进行分析,结合文献讨论该病的临床特征、遗传学特点及诊断。结果本例以眼眶绿色瘤起病的儿童急性髓系白血病(acute myeloid leukemia,AML)经血象、骨髓象确诊为M4Eo,染色体核型分析为40-45,XY,t(8:21)(q22,q22),常规化疗及大剂量阿糖胞苷治疗疗效欠佳。结论眼眶绿色瘤是急性髓性白血病的一种特殊临床表现,除常见于AML-M2型外,也可见于M4Eo;AML-M4Eo的染色体核型除了常见的inv(16)外,也可以是t(8;21)。通过对该例患儿的临床特点及治疗经过的分析,提示以眼眶绿色瘤起病、伴t(8;21)及亚二倍体核型改变的儿童M4Eo可能是一种预后不良的临床亚型。
Objective To report a case of M4Eo in children with orbital tumor of green tumor (t = 8; 21), and discuss the literature at home and abroad to improve the understanding of the disease. Methods The clinical manifestations, laboratory tests, diagnosis and treatment of this case were analyzed, and the clinical features, genetic characteristics and diagnosis of the disease were discussed in combination with the literature. Results In this case, children with ornithogalum neoplasia developed acute myeloid leukemia (AML). The bone marrow was diagnosed as M4Eo. The chromosome karyotype was 40-45, XY, t (8:21) (q22 , q22), conventional chemotherapy and high doses of cytarabine treatment ineffective. Conclusions The orbital tumor is a special clinical manifestation of acute myeloid leukemia. It can also be seen in M4Eo except AML-M2. The chromosomal karyotype of AML-M4Eo may be not only inv (16) but also t (8; 21). By analyzing the clinical features and treatment of this case, it is suggested that the onset of orbital tumor, M4Eo in children with t (8; 21) and subduploid karyotype may be a clinically sub-poor prognosis type.