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慢性粒单核细胞白血病:自然病程及预后决定因素

Chronic myelomonocytic leukemia: natural history and prognostic determinants.

作者信息

Tefferi A, Hoagland H C, Therneau T M, Pierre R V

机构信息

Division of Hematology, Mayo Clinic, Rochester, MN 55905.

出版信息

Mayo Clin Proc. 1989 Oct;64(10):1246-54. doi: 10.1016/s0025-6196(12)61287-7.

Abstract

A retrospective clinical review of 41 patients with chronic myelomonocytic leukemia revealed a median age of 66 years and a male:female ratio of 2.4:1. The disease was preceded by a myelodysplastic syndrome of a different subtype in 24% of the patients and transformed into acute leukemia in 24%. Splenomegaly was present in 54% of the patients and reached massive proportions in 24%. Chromosomal abnormalities occurred in 34% of those studied, most commonly in the younger age group; the most frequent were trisomy 8, monosomy 7, and deletions involving the long arms of chromosomes 20 and X. Polyclonal hypergammaglobulinemia was detected in 47% of the patients in whom serum protein electrophoresis was done. The median survival was 3 years. With use of univariate analysis, the statistically significant prognostic determinants were hemoglobin level, the "modified Bournemouth score," and bone marrow blast cell percentage. When these factors were subjected to a multivariate analysis, only bone marrow blast cell percentage was an independent prognostic determinant. Orally administered hydroxyurea controlled leukocytosis and splenomegaly in some patients without affecting the overall prognosis.

摘要

对41例慢性粒单核细胞白血病患者进行的回顾性临床研究显示,中位年龄为66岁,男女比例为2.4:1。24%的患者在患病前有不同亚型的骨髓增生异常综合征,24%的患者会转化为急性白血病。54%的患者出现脾肿大,24%的患者脾肿大达到巨大程度。34%的研究对象存在染色体异常,最常见于较年轻的年龄组;最常见的是8号染色体三体、7号染色体单体以及涉及20号和X号染色体长臂的缺失。在进行血清蛋白电泳的患者中,47%检测到多克隆高球蛋白血症。中位生存期为3年。单因素分析显示,具有统计学意义的预后决定因素是血红蛋白水平、“改良伯恩茅斯评分”和骨髓原始细胞百分比。对这些因素进行多因素分析时,只有骨髓原始细胞百分比是独立的预后决定因素。口服羟基脲可控制部分患者的白细胞增多和脾肿大,但不影响总体预后。

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