Kerkhofs H, Hermans J, Haak H L, Leeksma C H
Br J Haematol. 1987 Jan;65(1):73-81. doi: 10.1111/j.1365-2141.1987.tb06138.x.
The utility and prognostic significance of the FAB classification was studied in 237 patients with a myelodysplastic syndrome. No significant differences in actuarial survival and probability of transformation to acute leukaemia were found in patients with RA, AISA or CMML. The median survival time for the RA group was 50 months, for the AISA and CMML subclasses more than 60 months. The probability of transformation for the RA, AISA and CMML subgroups showed a linear trend with a probability of 25% for the RA, 16% for the AISA and of 18% for the CMML groups after a 5 year observation period. A uniformly poor prognosis was found for the RAEB and RAEB/t subgroups with median survival times of respectively 9 and 6 months. Chromosomal abnormalities were found in 68 out of 155 patients (44%). Patients with only normal metaphases or with abnormal metaphases together with karyotypic normal cells had a longer median survival time and a lower probability for transformation as compared to those with only abnormal metaphases. The most important factor in prognosis is the number of blast cells in blood and bone marrow. Age and sex, and certain quantitative and qualitative abnormalities in the peripheral blood appear of limited prognostic value for patients with RA, AISA and CMML. The longer life expectancy of 35 patients with CMML as compared to other series seems to be related to the percentage of blast cells at the time of diagnosis.
对237例骨髓增生异常综合征患者研究了FAB分类的实用性和预后意义。在难治性贫血(RA)、难治性贫血伴环形铁粒幼细胞增多(AISA)或慢性粒-单核细胞白血病(CMML)患者中,未发现精算生存率和转化为急性白血病概率的显著差异。RA组的中位生存时间为50个月,AISA和CMML亚类超过60个月。RA、AISA和CMML亚组的转化概率呈线性趋势,5年观察期后,RA组为25%,AISA组为16%,CMML组为18%。难治性贫血伴原始细胞过多(RAEB)和难治性贫血伴原始细胞过多转变型(RAEB/t)亚组的预后均较差,中位生存时间分别为9个月和6个月。155例患者中有68例(44%)发现染色体异常。与仅具有异常中期的患者相比,仅具有正常中期或具有异常中期以及核型正常细胞的患者中位生存时间更长,转化概率更低。预后的最重要因素是血液和骨髓中的原始细胞数量。年龄和性别以及外周血中的某些定量和定性异常对RA、AISA和CMML患者的预后价值有限。与其他系列相比,35例CMML患者较长的预期寿命似乎与诊断时原始细胞的百分比有关。