Demory J L, Dupriez B, Fenaux P, Laï J L, Beuscart R, Jouet J P, Deminatti M, Bauters F
Service des Maladies du Sang, Centre Hospitalier Régional, Lille, France.
Blood. 1988 Sep;72(3):855-9.
Cytogenetic analysis was performed in 47 newly diagnosed patients with agnogenic myeloid metaplasia (AMM); 32 had a normal karyotype (68%, group I), whereas 15 had clonal abnormalities (32%, group II). The most frequent abnormal findings were a 20q- deletion in six cases (either alone or within complex anomalies), interstitial 13q- deletion in three cases (and monosomy 13 in one case), and acquired trisomy 21 or 21p+ in three cases. Four cases exhibited complex aberrations involving several chromosomes, sometimes with a mosaicism. In two patients with an initial abnormal karyotype, further cytogenetic analysis during the disease course showed the appearance of additional clonal anomalies, and particularly of a probable Philadelphia (Ph1) variant in one case. Treatment was essentially supportive. Survival was significantly shorter in group II (median, 30 months) compared with group I (median, not reached at 6 years; P = .015). In univariate analysis, other parameters significantly associated with a poor prognosis (P less than .05) were higher age, anemia, and increased percentage of circulating blasts. However, in a multivariate analysis, only cytogenetic abnormalities and age retained their independent prognostic value.
对47例新诊断的原发性骨髓化生（AMM）患者进行了细胞遗传学分析；32例核型正常（68%，I组），而15例有克隆性异常（32%，II组）。最常见的异常发现是6例20号染色体长臂缺失（单独出现或存在于复杂异常中），3例13号染色体间质缺失（1例为13号染色体单体），3例获得性21三体或21号染色体短臂增加。4例表现出涉及多条染色体的复杂畸变，有时伴有嵌合体。2例初始核型异常的患者在病程中进一步的细胞遗传学分析显示出现了额外的克隆性异常，特别是1例可能的费城（Ph1）变异型。治疗主要是支持性的。II组的生存期（中位数30个月）明显短于I组（中位数，6年时未达到；P = 0.015）。单因素分析中，与预后不良显著相关（P < 0.05）的其他参数包括年龄较大、贫血和循环原始细胞百分比增加。然而，多因素分析中，只有细胞遗传学异常和年龄保留了其独立的预后价值。
