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骨髓增生异常综合征:根据FAB分类法对101例病例的研究。

Myelodysplastic syndromes: a study of 101 cases according to the FAB classification.

作者信息

Vallespi T, Torrabadella M, Julia A, Irriguible D, Jaen A, Acebedo G, Triginer J

出版信息

Br J Haematol. 1985 Sep;61(1):83-92. doi: 10.1111/j.1365-2141.1985.tb04063.x.

Abstract

The myelodysplastic syndromes (MDS) are a group of closely related disorders characterized by chronic cytopenias with cellular marrow, poor prognosis and refractoriness to treatment. We studied 101 consecutive cases of MDS diagnosed over a 7-year period. Peripheral blood (PB) and bone marrow (BM) samples were reviewed and classified according to the proposals of the French-American-British (FAB) cooperative group for MDS. The combined analysis of the initial laboratory features and qualitative haematological abnormalities readily allowed the distinction between the different subgroups. Thirty-two of 79 cases (40.5%) evolved towards other diseases, frequently acute leukaemia (24/79, 30%), or transformed into other MDS (7/79, 9%). In five cases, initially classified as refractory anaemia (RA) or refractory anaemia with ring sideroblasts (RAS), a transitory change to another type of MDS--two chronic myelomonocytic leukaemias (CMML), two refractory anaemias with excess of blasts (RAEB) and one refractory anaemia with excess of blasts 'in transformation' (RAEB-t)--was observed before the evolution towards acute leukaemia. This provides a new link between all these syndromes and increases the number of transitions to other MDS. Overall prognosis was very poor, with differences between subgroups. RA had the best prognosis whereas RAEB-t had the worst one. This study shows that the FAB classification is readily usable and defines well-characterized subgroups of MDS, although there are frequent transitional forms, and as the natural history of the MDS unfolds they may convert into another. The actual poor prognosis and the frequent evolution towards acute leukaemia makes necessary to investigate new methods of treatment for these disorders.

摘要

骨髓增生异常综合征(MDS)是一组密切相关的疾病,其特征为骨髓细胞增多的慢性血细胞减少、预后不良及治疗抵抗。我们研究了7年间连续诊断的101例MDS病例。根据法国-美国-英国(FAB)协作组对MDS的提议,对外周血(PB)和骨髓(BM)样本进行了复查和分类。对初始实验室特征和定性血液学异常的综合分析很容易区分不同的亚组。79例中有32例(40.5%)演变为其他疾病,常见的是急性白血病(24/79,30%),或转变为其他MDS(7/79,9%)。在5例最初被分类为难治性贫血(RA)或环形铁粒幼细胞性难治性贫血(RAS)的病例中,在演变为急性白血病之前,观察到向另一种类型的MDS的短暂转变——2例慢性粒-单核细胞白血病(CMML)、2例原始细胞过多的难治性贫血(RAEB)和1例“转变中”原始细胞过多的难治性贫血(RAEB-t)。这在所有这些综合征之间建立了新的联系,并增加了向其他MDS转变的数量。总体预后非常差,亚组之间存在差异。RA预后最好,而RAEB-t预后最差。这项研究表明FAB分类易于使用,并能很好地定义MDS的特征明确的亚组,尽管存在频繁的过渡形式,并且随着MDS自然病程的发展,它们可能会转变为另一种类型。实际的不良预后以及向急性白血病的频繁演变使得有必要研究这些疾病的新治疗方法。

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