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原发性骨髓增生异常综合征患者克隆性染色体异常的预后价值

Prognostic value of clonal chromosomal abnormalities in patients with primary myelodysplastic syndromes.

作者信息

Gyger M, Infante-Rivard C, D'Angelo G, Forest L, Lussier P

机构信息

Department of Hematology, Maisonneuve-Rosemont Hospital, Montreal, Quebec, Canada.

出版信息

Am J Hematol. 1988 May;28(1):13-20. doi: 10.1002/ajh.2830280104.

Abstract

Chromosome analyses were carried out on bone marrow cells from 43 consecutive patients with primary myelodysplastic syndromes (MDS), classified according to the French-American-British (FAB) cooperative group criteria. The objective was to evaluate the prognostic value of clonal chromosomal abnormalities and of an excess of blasts for early death from acute nonlymphocytic leukemia (ANLL) and/or bone marrow failure (BMF). Patients were subdivided into two main groups: (1) refractory anemia without an excess of blasts (RAWEB), grouping patients with refractory anemia (RA) and refractory anemia with ringed sideroblasts (RARS), and (2) refractory anemia with an excess of blasts (RAEB), grouping patients with refractory anemia with an excess of blasts (RAEB) and refractory anemia with an excess of blasts in transformation (RAEBt). There were 29 patients with RAWEB and 14 with RAEB. The median time of observation was 26 months for RAWEB and 12 months for RAEB. Ten RAWEB patients (34%) and 11 RAEB patients (78%) had clonal chromosomal abnormalities. Among the ten RAWEB patients with clonal abnormalities, one (10%) died from ANLL, while of 19 RAWEB patients with a normal karyotype, two (10%) died from ANLL or BMF. The median survival for patients with RAWEB and an abnormal karyotype was not reached. In contrast, eight of the 11 RAEB patients with clonal chromosomal abnormalities (74%) died from ANLL or BMF. The median survival in this sub-group was 7 months. By using a Cox proportional hazard regression analysis, it was determined that a karyotype abnormality was not a significant predictory of survival once the contribution of the RAWEB/RAEB variable was taken into account. Being in the RAEB group was associated with a relative risk of 10.6 of dying from ANLL or BMF (beta = 2.36, standard error (SE) = 0.68, P = .0001). We conclude that classifying patients according to an excess of blasts will lead to a better prediction of survival than determining karyotype abnormality.

摘要

对43例连续性原发性骨髓增生异常综合征(MDS)患者的骨髓细胞进行了染色体分析,这些患者根据法美英(FAB)协作组标准进行分类。目的是评估克隆性染色体异常和原始细胞增多对急性非淋巴细胞白血病(ANLL)和/或骨髓衰竭(BMF)导致早期死亡的预后价值。患者被分为两个主要组:(1)无原始细胞增多的难治性贫血(RAWEB),包括难治性贫血(RA)和环形铁粒幼细胞性难治性贫血(RARS)患者;(2)有原始细胞增多的难治性贫血(RAEB),包括有原始细胞增多的难治性贫血(RAEB)和转化中有原始细胞增多的难治性贫血(RAEBt)患者。有29例RAWEB患者和14例RAEB患者。RAWEB患者的中位观察时间为26个月,RAEB患者为12个月。10例RAWEB患者(34%)和11例RAEB患者(78%)有克隆性染色体异常。在10例有克隆性异常的RAWEB患者中,1例(10%)死于ANLL,而在19例核型正常的RAWEB患者中,2例(10%)死于ANLL或BMF。有异常核型的RAWEB患者的中位生存期未达到。相比之下,11例有克隆性染色体异常的RAEB患者中有8例(74%)死于ANLL或BMF。该亚组的中位生存期为7个月。通过Cox比例风险回归分析确定,一旦考虑到RAWEB/RAEB变量的影响,核型异常并不是生存的显著预测指标。处于RAEB组与死于ANLL或BMF的相对风险为10.6相关(β=2.36,标准误(SE)=0.68,P=.0001)。我们得出结论,根据原始细胞增多对患者进行分类比确定核型异常能更好地预测生存。

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