Shin K, Kikukawa M, Yoneda Y, Uno M, Iwamoto T, Takasaki M
Department of Geriatric Medicine, Tokyo Medical College.
Nihon Ronen Igakkai Zasshi. 1996 Sep;33(9):702-6. doi: 10.3143/geriatrics.33.702.
An 80-year-old man with leukocytosis was admitted to our hospital on February 20, 1995. There were no blasts in the peripheral blood, but leukocytosis, associated with marked monocytosis, anemia, and thrombocytopenia was observed. The bone marrow was hyperplastic and there was a slight decrease in megakaryocytes. The myelogram showed 5.6% blasts + promyelocytes, and there was no basophilia. Mild dysplasia of third-line cells was observed. Chromosome analysis yielded 47, XY, +8, t (9;22) (q34;q11), and the Philadelphia (Ph1) chromosome and trisomy 8 were detected. Fluorescence in-situ hybridization revealed a large ber. Because the French-American-British (FAB) cooperative group diagnostic criteria for chronic myelomonocytic leukemia (CMMoL) were fulfilled and the Ph1 chromosome was detected, a diagnosis of Ph1-positive CMMoL was made. Hydroxyurea was given for cytoreduction and the patient has been followed with no evidence of acute transformation for about one year. CMMoL is classified as a myelodysplastic syndrome. However, it is rare to find the Ph1 chromosome in patients with these syndromes, and there have been very few reports of its detection in CMMoL. CMMoL may not be a single disease entity, and it may be useful to investigate more cases like the present one, to reassess this disease.
一名80岁白细胞增多症男性患者于1995年2月20日入院。外周血中未见原始细胞，但观察到白细胞增多，并伴有明显的单核细胞增多、贫血和血小板减少。骨髓增生，巨核细胞略有减少。骨髓象显示原始细胞+早幼粒细胞占5.6%，无嗜碱性粒细胞增多。观察到三线细胞轻度发育异常。染色体分析结果为47，XY，+8，t（9；22）（q34；q11），检测到费城（Ph1）染色体和8号染色体三体。荧光原位杂交显示有一个大的ber。由于符合法国-美国-英国（FAB）协作组慢性粒单核细胞白血病（CMMoL）的诊断标准且检测到Ph1染色体，故诊断为Ph1阳性CMMoL。给予羟基脲进行细胞减灭治疗，患者已随访约一年，无急性转化迹象。CMMoL被归类为骨髓增生异常综合征。然而，在这些综合征患者中发现Ph1染色体的情况很少见，在CMMoL中检测到该染色体的报道也非常少。CMMoL可能不是单一的疾病实体，研究更多像本病例这样的病例以重新评估这种疾病可能会有帮助。
