Department of Developmental Medicine (Pediatrics), Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan.
Int J Hematol. 2010 Apr;91(3):525-9. doi: 10.1007/s12185-010-0504-1. Epub 2010 Feb 10.
Myeloid/NK cell precursor lymphoma/leukemia has been suggested to be of precursor NK origin. We report a 1-year-old boy with myeloid/NK cell precursor lymphoma/leukemia who presented with a skin nodule. Biopsy of the skin nodule specimen revealed CD45(+), CD56(+), myeloid antigen(+), CD7(-), CD3(-), CD19(-), CD34(-), CD4(-) by flow cytometry and myeloperoxidase (MPO)(+), TdT(+), cytoplasmic CD3(+) by immunohistochemistry. He received acute myeloid leukemia-oriented combination chemotherapy. Although his skin nodule disappeared, regrowth was observed; a biopsy was performed again, showing that the immunophenotype was CD56(+), myeloid antigen(+), HLA-DR(+), MPO(-), CD3(+) and TCRgammadelta(+). Being refractory to chemotherapy, he underwent stem cell transplantation from his father. However, he relapsed again and died from septic shock. Although myeloid/NK cell precursor acute leukemia is characterized by CD7(+), CD56(+), CD3(-), CD34(+) and myeloid antigen(+) phenotype, the blast cells of our patients lacked CD34 and CD7 expression while expressing myeloid antigens. Furthermore, CD94 1A transcripts were predominantly expressed rather than CD94 1B, implying their origin in immature NK cells. After acute myeloid leukemia-oriented combination chemotherapy, his blasts acquired stable CD3 expression and TCRgammadelta rearrangement at recurrence. The blast cells possessed features overlapping both myeloid/NK precursor acute leukemia and blastic NK/precursor acute lymphoma/leukemia. These observations suggest that two subclones originated from common precursors.
髓系/NK 细胞前体细胞淋巴瘤/白血病被认为起源于前体细胞 NK 细胞。我们报告了一例 1 岁男性患儿，以皮肤结节为首发表现，诊断为髓系/NK 细胞前体细胞淋巴瘤/白血病。皮肤结节活检标本的流式细胞术结果显示 CD45(+)、CD56(+)、髓系抗原(+)、CD7(-)、CD3(-)、CD19(-)、CD34(-)、CD4(-)，免疫组化结果显示髓过氧化物酶(MPO)(+)、TdT(+)、细胞质 CD3(+)。患儿接受了急性髓系白血病为导向的联合化疗。虽然皮肤结节消退，但再次出现结节，再次行皮肤结节活检，免疫表型为 CD56(+)、髓系抗原(+)、HLA-DR(+)、MPO(-)、CD3(+)和 TCRgammadelta(+)。患儿对化疗耐药，接受了来自父亲的造血干细胞移植。然而，他再次复发并死于感染性休克。尽管髓系/NK 细胞前体急性白血病的特征为 CD7(+)、CD56(+)、CD3(-)、CD34(+)和髓系抗原(+)，但该患儿的原始细胞缺乏 CD34 和 CD7 表达，而表达髓系抗原。此外，CD94 1A 转录本主要表达而不是 CD94 1B，提示其起源于不成熟 NK 细胞。急性髓系白血病为导向的联合化疗后，复发时患儿的原始细胞获得稳定的 CD3 表达和 TCRgammadelta 重排。原始细胞具有髓系/NK 前体急性白血病和原始 NK/前体急性淋巴瘤/白血病的重叠特征。这些观察结果提示两个亚克隆起源于共同的前体。
