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初发急性髓系白血病中三系发育异常的意义:临床和实验室特征

The significance of trilineage myelodysplasia in de novo acute myeloblastic leukemia: clinical and laboratory features.

作者信息

Lima C S, Vassalo J, Lorand-Metze I, Bechelli A P, Souza C A

机构信息

Hematology and Hemotherapy Center, Faculty of Medical Sciences, State University of Campinas, Sao Paulo, Brazil.

出版信息

Haematologia (Budap). 1997;28(2):85-95.

PMID:9283908
Abstract

A prospective study was undertaken to elucidate the clinical and laboratory differences between de novo acute myeloid leukemia (AML) and AML with trilineage myelodysplasia (AML-TMDS). One hundred and seven patients with AML were diagnosed at the University Hospital between January 1987 and July 1992, and were followed until July 1995. TMDS was identified in 17 of them (16%). With regard to age and sex distribution no difference was found between AML patients with and without TMDS (p = 0.43, p = 0.54, respectively). The duration of symptoms at presentation in AML-TMDS was similar to those observed in de novo AML (p = 0.29). Hemoglobin values and platelet counts were similar in both groups of patients (p = 0.45, p = 0.44, respectively). However, peripheral white blood cell and neutrophil counts, as well as blast counts in AML-TMDS patients were lower than those observed in AML without TMDS patients (p < 0.001 for all of them). Bone marrow blast counts in de novo AML were higher than the values observed in AML-TMDS patients (p < 0.001). TMDS occurred predominantly in M2 and M6 FAB types, and was absent in the M3 type. Bone marrow histology showed no particular feature that could be of diagnostic relevance. The remission rates were similar in both groups of patients (p = 0.55). The same was true for the probability of disease-free survival and overall survival during the period of study (p = 0.50, p = 0.33, respectively). These results suggest that: 1) in AML-TMDS patients, leukemia transformation occurs in a more undifferentiated pluripotent stem cell, leading to a dysplastic residual hemopoiesis besides the blast proliferation; 2) the incidence of TMDS in our group of patients did not influence the clinical outcome after treatment of the disease.

摘要

开展了一项前瞻性研究,以阐明初发性急性髓系白血病(AML)与伴有三系骨髓发育异常的AML(AML-TMDS)之间的临床和实验室差异。1987年1月至1992年7月期间,在大学医院诊断出107例AML患者,并随访至1995年7月。其中17例(16%)被确诊为AML-TMDS。在年龄和性别分布方面,伴有和不伴有TMDS的AML患者之间未发现差异(p值分别为0.43和0.54)。AML-TMDS患者出现症状的持续时间与初发性AML患者相似(p = 0.29)。两组患者的血红蛋白值和血小板计数相似(p值分别为0.45和0.44)。然而,AML-TMDS患者的外周血白细胞和中性粒细胞计数以及原始细胞计数低于不伴有TMDS的AML患者(所有p值均<0.001)。初发性AML患者的骨髓原始细胞计数高于AML-TMDS患者(p < 0.001)。TMDS主要发生在FAB分型的M2和M6型,M3型中不存在。骨髓组织学未显示出具有诊断相关性的特殊特征。两组患者的缓解率相似(p = 0.55)。在研究期间,无病生存率和总生存率的情况也是如此(p值分别为0.50和0.33)。这些结果表明:1)在AML-TMDS患者中,白血病转化发生在更未分化的多能干细胞中,除了原始细胞增殖外还导致发育异常的残留造血;2)我们这组患者中TMDS的发生率不影响疾病治疗后的临床结局。

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