Nagafuji K, Iwakiri R, Miyamoto T, Okamura H, Yokota E, Matsumoto I
Department of Internal Medicine, Matsuyama Red Cross Hospital.
Rinsho Ketsueki. 1992 Sep;33(9):1226-30.
The authors report a rare case of chronic myelogenous leukemia (CML) in which the Ph1 clone disappeared after remission induction of lymphoid crisis. A 58-year-old man was admitted to our hospital because of fever in July 1988. The white cell count was elevated. Bone marrow aspirate showed hypercellularity with myeloid hyperplasia. In the chromosomal analysis, Ph1 chromosomes were detected in 100% of bone marrow cells analysed. Diagnosis of CML was made and treatment was initiated with recombinant interferon-alpha 2a. Hematological remission without cytogenetic improvement was achieved. In March 1990 he developed lymphoid crisis with proliferation of CD10-positive cells. The chromosomal analysis revealed additional abnormalities including, 45, X, -Y, t(9;22) (q34;q11), +1, -8. With vincristine 0.6 mgX4, pirarubicin 15 mgX4, dexamethasone 40 mgX4 therapy complete remission was obtained. In December 1990 the Ph1 positive clone completely disappeared judging from normal karyotypes in the chromosomal analysis and the disappearance of M-bcr gene rearrangement.
作者报告了一例罕见的慢性粒细胞白血病(CML)病例,该病例中费城染色体(Ph1)克隆在淋巴细胞危象缓解诱导后消失。一名58岁男性因发热于1988年7月入院。白细胞计数升高。骨髓穿刺显示细胞增多伴髓系增生。染色体分析显示,在分析的100%骨髓细胞中检测到Ph1染色体。诊断为CML,并开始用重组干扰素α-2a进行治疗。实现了血液学缓解但细胞遗传学未改善。1990年3月,他出现了伴有CD10阳性细胞增殖的淋巴细胞危象。染色体分析显示有其他异常,包括45,X,-Y,t(9;22)(q34;q11),+1,-8。通过长春新碱0.6mg×4、吡柔比星15mg×4、地塞米松40mg×4治疗获得了完全缓解。1990年12月,根据染色体分析中的正常核型以及M-bcr基因重排的消失判断,Ph1阳性克隆完全消失。