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[嗜酸性粒细胞增多综合征发病12年后发生的CD19阳性急性髓细胞白血病]

[CD19-positive acute myeloblastic leukemia developed 12 years after the onset of hypereosinophilic syndrome].

作者信息

Handa T, Yamamoto K, Tadokoro J, Kikkawa Y, Tsurumi S, Nakamura Y, Saito K, Uzuka Y, Saito Y, Furusawa S

机构信息

Department of Hematology, Dokkyo University School of Medicine.

出版信息

Rinsho Ketsueki. 2000 Sep;41(9):723-8.

Abstract

We report a rare case of hypereosinophilic syndrome (HES) that developed to acute myeloblastic leukemia (AML). The patient, a 34-year-old man, presented with eosinophilia of unknown origin (white blood cells 38,200/microliter with 74% eosinophils) and pericardial effusion, and was diagnosed as having HES with a normal karyotype. He received four cycles of combination chemotherapy including cyclophosphamide, cytosine arabinoside and vindesine, and thereafter remained in remission. After 12 years, he was referred to our hospital because of fever and malaise. On admission, CBC showed white blood cells 3,000/microliter with 70% myeloblasts and 3% eosinophils. The bone marrow was hypercellular with 95% blasts, which were negative for myeloperoxidase (MPO) staining. Immunophenotype analysis revealed that the cells were positive for CD13, CD19, CD34, HLA-DR and cytoplasmic MPO. CD19-positive AML was diagnosed. Cytogenetic analysis showed 46, XY, t(6;21)(q13;q22), add(7)(q11) in 19 of 20 metaphase spreads. Rearrangement of the AML1 gene at 21q22 and fusion of the BCR/ABL gene could not be detected by fluorescence in situ hybridization analysis. The patient received combination chemotherapy and achieved a complete remission. Chromosome aberrations involving 7q as well as 21q22 suggested that the initial chemotherapy for HES might have been implicated in the pathogenesis of acute leukemia in this case.

摘要

我们报告了一例罕见的高嗜酸性粒细胞综合征(HES)发展为急性髓系白血病(AML)的病例。患者为一名34岁男性,表现为不明原因的嗜酸性粒细胞增多(白细胞计数38,200/微升,嗜酸性粒细胞占74%)及心包积液,被诊断为核型正常的HES。他接受了四个周期的联合化疗,包括环磷酰胺、阿糖胞苷和长春地辛,此后病情缓解。12年后,因发热和不适转诊至我院。入院时,血常规显示白细胞计数3,000/微升,原始粒细胞占70%,嗜酸性粒细胞占3%。骨髓增生极度活跃,原始细胞占95%,髓过氧化物酶(MPO)染色阴性。免疫表型分析显示细胞CD13、CD19、CD34、HLA - DR及胞质MPO阳性。诊断为CD19阳性AML。细胞遗传学分析显示,20个中期分裂相中19个为46, XY, t(6;21)(q13;q22), add(7)(q11)。荧光原位杂交分析未检测到21q22处AML1基因重排及BCR/ABL基因融合。患者接受联合化疗后达到完全缓解。涉及7q以及21q22的染色体畸变提示,该病例中HES的初始化疗可能与急性白血病的发病机制有关。

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