Economopoulos T, Stathakis N, Foudoulakis A, Papadoulis N, Dervenoulas J, Papageorgiou E, Anastassiou C, Hadjioannou J, Raptis S
Eur J Haematol. 1987 Apr;38(4):338-44. doi: 10.1111/j.1600-0609.1987.tb00008.x.
The clinical and haematological findings in 131 patients with myelodysplastic syndromes (MDS), none of which had previously received chemotherapy or radiotherapy, classified according to the FAB criteria, were analysed. The distribution among the 5 subgroups was: RA 31 patients, RAS 19, RAEB 23, CMML 29 and RAEBT 29 patients. There were difficulties in the classification of 24 patients. These included, first, 8 cases with myeloid hyperplasia of the bone marrow (BM) but without monocytosis or excess of blasts of the BM. They were classified as RA. Second, 8 cases with sideroblastosis but with monocytosis or excess of blasts of the BM were classified 3 as RAEB, 2 as CMML and 3 as RAEBT. Finally, 8 cases with absolute monocytosis and BM blasts 15-30% were classified as CMML. 37 of 82 dead patients (45.1%) had transformed to acute non-lymphoblastic leukaemia (ANLL). The incidence of evolution to ANLL was low for RA and RAS (6.30% and 12.5% respectively), while it was 37.5% for RAEB, 57.1% for CMML and 77.2% for RAEBT. The median survival for each subgroup was: RA 18 months; RAS 25; RAEB 13; CMML 14 and RAEBT 10 months. It is concluded that the FAB classification with some modifications recognises group of MDS with different prognosis.
对131例骨髓增生异常综合征(MDS)患者的临床和血液学检查结果进行了分析,这些患者之前均未接受过化疗或放疗,且根据FAB标准进行了分类。5个亚组的分布情况为:难治性贫血(RA)31例,环形铁粒幼细胞性难治性贫血(RAS)19例,难治性贫血伴原始细胞增多(RAEB)23例,慢性粒-单核细胞白血病(CMML)29例,难治性贫血伴原始细胞增多-转变型(RAEBT)29例。有24例患者的分类存在困难。其中,首先,8例骨髓(BM)有髓系增生但无单核细胞增多或BM原始细胞增多的患者被分类为RA。其次,8例有铁粒幼细胞增多但伴有单核细胞增多或BM原始细胞增多的患者,3例被分类为RAEB,2例被分类为CMML,3例被分类为RAEBT。最后,8例有绝对单核细胞增多且BM原始细胞为15%-30%的患者被分类为CMML。82例死亡患者中有37例(45.1%)已转化为急性非淋巴细胞白血病(ANLL)。向ANLL演变的发生率在RA和RAS中较低(分别为6.30%和12.5%),而在RAEB中为37.5%,在CMML中为57.1%,在RAEBT中为77.2%。每个亚组的中位生存期为:RA 18个月;RAS 25个月;RAEB 13个月;CMML 14个月;RAEBT 10个月。结论是,经过一些修改的FAB分类法可识别出预后不同的MDS组。