Ota S, Tanaka J, Kobayashi S, Tsuda Y, Mori A, Noto S, Yamamoto Y, Hashino S, Musashi M, Imamura M, Asaka M
Third Department of Internal Medicine, Hokkaido University School of Medicine, Kita-15, Nishi-7, Kita-ku, Sapporo, 060-8648, Japan.
Acta Haematol. 2000;104(4):207-11. doi: 10.1159/000046517.
We experienced the case of an 82-year-old man with chronic neutrophilic leukemia (CNL) with dysplastic features in the granulocytic lineage which subsequently progressed to acute myeloblastic leukemia (AML) with myelofibrosis. The patient had hepatosplenomegaly, but there was no evident cause of neutrophilic leukocytosis. The cytogenetic study showed that he had a normal karyotype. Concentrations of the serum granulocyte colony-stimulating factor (G-CSF) were not detectable. Two years after the diagnosis of CNL, blastic transformation to AML occurred with myelofibrosis and significant morphological abnormalities in neutrophils. The blasts were positive for myeloperoxidase, CD33, CD34, and HLA-DR, and the presence of dysplasia within the granulocytic lineage suggested that he had an abnormality at the level of the granulocyte-committed progenitors. Heterogeneous origins of CNL might lead to various clinicopathological features in each case.
我们遇到过一例82岁男性慢性中性粒细胞白血病(CNL)患者,其粒细胞系具有发育异常特征,随后进展为伴有骨髓纤维化的急性髓细胞白血病(AML)。该患者有肝脾肿大,但中性粒细胞增多症无明显病因。细胞遗传学研究显示其核型正常。血清粒细胞集落刺激因子(G-CSF)浓度检测不到。在诊断为CNL两年后,发生了向AML的原始细胞转化,伴有骨髓纤维化和中性粒细胞明显的形态学异常。原始细胞髓过氧化物酶、CD33、CD34和HLA-DR呈阳性,粒细胞系内发育异常的存在提示他在粒细胞定向祖细胞水平存在异常。CNL的异质性起源可能导致每个病例出现各种临床病理特征。