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[骨髓增生异常综合征的演变性流行病学特征(1959 - 1993年)。与急性髓系白血病和再生障碍性全血细胞减少症的比较研究]

[Evolutive epidemiologic profile of myelodysplastic syndromes (1959-1993). Comparative study with acute myeloid leukemia and aplastic pancytopenias].

作者信息

Sánchez Fayos J, Outeiriño Pérez J J, Prieto E, Román A, Olavarría E, Cabello A, Soto C, Calabuig T, Paniagua C, Sanz B

机构信息

Servicio de Hematología y Hemoterapia, Fundación Jiménez Díaz, Facultad de Medicina, Universidad Autónoma, Madrid.

出版信息

Sangre (Barc). 1994 Dec;39(6):441-8.

PMID:7855696
Abstract

PURPOSE

To compare the maximum epidemiologic data attained from myelodysplastic syndromes (MDS) with those of two main panmyelopathies, namely acute myeloblastic leukaemia (AML) and aplastic pancytopenia (AP).

PATIENTS AND METHODS

A retrospective analysis was carried out on 21,135 patients included in the Bone Marrow Study Registry of the Jiménez Díaz Foundation along 35 years (1959-1993). The data were grouped into seven five-year periods. Of these, in the first three the study was performed on bone-marrow aspirates; after 1976 the histopathological study of bone-marrow biopsies was introduced, and since 1979 the karyotype has been regularly examined. The MDS were classified in accordance with the FAB system. With these premises borne in mind, the following aspects were considered: diagnostic interpretation of MDS along the years; diagnostic incidence of MDS, AML and AP in each of the five-year periods; relative frequency of those diagnosis with respect to the total number of cases; evolutive profile of sex and age at diagnosis; quantitative significance of secondary MDS-AL and toxic AP along the years; MDS subtypes and their epidemiologic characteristics.

RESULTS

A total of 510 MDS, 610 AML and 223 AP cases were identified. With respect to the sex of the MDS patients, some changes have been seen along the years, from an M/F ratio of 1.9 to 1.0; and the mean age at diagnosis raised from 53.3 years (with only 1.7% of the cases over 65 years of age) to 71.4 years (with 76.9% of the cases over 65 years of age), all this within the 1959-1989 period. The incidence of AML and AP has remained stable for the last 20 years; on the contrary, MDS have been increasing continuously along the 35 years of the study, which poses for a higher number of new cases in every period (from 35 to 119) and also for a higher relative frequency in the registry (from 1.37% to 4.40%) within the period 1959-1989. Valuable toxic history was progressively increasing in secondary MDS-AML and progressively decreasing in AP. With respect to the FAB subtypes of MDS, and taking into account the last of the five-year periods, the most frequently diagnosed were RA and RSA followed by RAEB, CMML and RAEB-T.

CONCLUSIONS

The increment of the incidence of MDS cases correlates significantly with the increment of the patients aged over 65 years. This incidence appears to be scarcely influenced by previous mutagenic agents (radiotherapy, chemotherapy) and might be due to a better understanding of MDS.

摘要

目的

比较骨髓增生异常综合征(MDS)所获得的最大流行病学数据与两种主要全血细胞减少症,即急性髓细胞白血病(AML)和再生障碍性全血细胞减少症(AP)的相关数据。

患者与方法

对希门尼斯·迪亚兹基金会骨髓研究登记处35年(1959 - 1993年)纳入的21135例患者进行回顾性分析。数据被分为七个五年期。其中,在前三个五年期,研究是基于骨髓穿刺液进行的;1976年后引入了骨髓活检的组织病理学研究,自1979年起定期检查核型。MDS根据FAB系统进行分类。在铭记这些前提的情况下,考虑了以下方面:多年来MDS的诊断解读;每个五年期内MDS、AML和AP的诊断发病率;这些诊断相对于病例总数的相对频率;诊断时的性别和年龄演变情况;多年来继发性MDS - AL和中毒性AP的定量意义;MDS亚型及其流行病学特征。

结果

共识别出510例MDS、610例AML和223例AP病例。关于MDS患者的性别,多年来有一些变化,男女比例从1.9变为1.0;诊断时的平均年龄从53.3岁(65岁以上病例仅占1.7%)提高到71.4岁(65岁以上病例占76.9%),所有这些变化均发生在1959 - 1989年期间。AML和AP的发病率在过去20年一直保持稳定;相反,在35年的研究期间,MDS一直在持续增加,这导致每个时期新病例数量增加(从35例增至119例),并且在登记处的相对频率也更高(从1.37%增至4.40%),这一情况同样出现在1959 - 1989年期间。继发性MDS - AML中有价值的中毒史逐渐增加,而AP中的中毒史逐渐减少。关于MDS的FAB亚型,考虑到最后一个五年期,最常诊断出的是RA和RSA,其次是RAEB、CMML和RAEB - T。

结论

MDS病例发病率的增加与65岁以上患者数量的增加显著相关。这种发病率似乎几乎不受先前诱变剂(放疗、化疗)的影响,可能是由于对MDS有了更好的认识。

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