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成人慢性和亚急性粒单核细胞白血病:60例报告并特别提及预后因素

Chronic and subacute myelomonocytic leukaemia in the adult: a report of 60 cases with special reference to prognostic factors.

作者信息

Fenaux P, Jouet J P, Zandecki M, Lai J L, Simon M, Pollet J P, Bauters F

出版信息

Br J Haematol. 1987 Jan;65(1):101-6. doi: 10.1111/j.1365-2141.1987.tb06142.x.

Abstract

We report 60 cases of chronic and subacute myelomonocytic leukaemias (CMML and SMML) in the adult, using the FAB group criteria. The M/F sex ratio was 3.3 and the mean age 67.5 years. Splenomegaly was found in 32% of cases, hyperleucocytosis in 52% of cases and mean blood monocytosis was 4.3 X 10(9)/l. Marrow smears showed an excess of blasts in 57% of patients, a moderate increase in monocytes in most cases and frequent myelodysplastic features. An increase in serum lysozyme and polyclonal hypergammaglobulinaemia were usual and clonal cytogenetic anomalies found in about half of the patients tested. Treatment was usually palliative and the median survival was 28 months, a blastic transformation being responsible for a third of the deaths. Prognostic factors at diagnosis were analysed retrospectively in the 46 patients who had sufficient follow up. Percentage of marrow blasts haemoglobin level and blood monocytosis at diagnosis, were subject to multivariate analysis, resulting in a discriminant 'score'. This allowed assignment of each patient into one of two prognostic subgroups (10.9% probability of error): a poor prognosis one, with a life expectancy of less than 1 year and a high risk of acute transformation (subgroup termed SMML) and a better prognosis subgroup (termed CMML), with some CMML patients surviving over 5 years.

摘要

我们采用FAB组标准报告了60例成人慢性和亚急性粒单核细胞白血病(CMML和SMML)。男女比例为3.3,平均年龄67.5岁。32%的病例发现脾肿大,52%的病例有白细胞增多,平均血单核细胞增多为4.3×10⁹/L。骨髓涂片显示57%的患者原始细胞增多,大多数病例单核细胞中度增加且常有骨髓发育异常特征。血清溶菌酶升高和多克隆高球蛋白血症常见,约一半接受检测的患者发现有克隆性细胞遗传学异常。治疗通常是姑息性的,中位生存期为28个月,三分之一的死亡由原始细胞转化所致。对46例有足够随访资料的患者进行回顾性分析诊断时的预后因素。对诊断时骨髓原始细胞百分比、血红蛋白水平和血单核细胞增多情况进行多变量分析,得出一个判别“分数”。这使得可将每位患者归入两个预后亚组之一(错误概率为10.9%):预后不良组,预期寿命不到1年且急性转化风险高(称为SMML亚组);预后较好亚组(称为CMML),一些CMML患者存活超过5年。

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