Update on the prognostic implication of morphology, histology, and karyotype in primary myelodysplastic syndromes.

Hematologic pathology Pub Date : 1991-01-01
G Verhoef, C De Wolf-Peeters, S Kerim, J Van De Broeck, C Mecucci, H Van den Berghe, M Boogaerts
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Abstract

The prognostic value of the FAB classification, bone marrow histology, Bournemouth score, and chromosome findings was studied in 88 patients with primary myelodysplastic syndromes. The median survival for the whole group of patients was 22 months (RA 61.7 months, RARS 31.6 months, CMML 15.7 months, RAEB 10.3 months, and RAEBt 8.2 months). Chromosomal abnormalities were found in 37 of the 70 patients investigated (52%). Only the differences in survival between patients with complex versus normal karyotype were statistically significant (p = 0.02). The presence of small blastic cells, located away from the endosteal surface (abnormal localization of immature blasts or ALIP) appears to be a major prognostic factor in predicting the duration of survival and progression to ANLL, especially in the FAB subgroups RA and RARS. Median survival for the 22 ALIP- cases with RA/RARS was 65 months, compared with 31 months for the ALIP+ cases (p = 0.0006). Nine ALIP+ patients (53%) developed ANLL in contrast to 3 (13%) of the ALIP- cases (p = 0.008). By redefining ALIP and evaluating the number and characteristics of the accompanying cells, histological subtypes were distinguished correlating largely with the FAB subgroups. Our findings demonstrate the prognostic importance of bone marrow biopsy and quantifying the complexity of bone marrow chromosome changes. It should be helpful in evaluating current attempts to find effective treatment for patients with MDS.

原发性骨髓增生异常综合征的形态学、组织学和核型预后意义的最新进展。
研究了88例原发性骨髓增生异常综合征患者的FAB分型、骨髓组织学、伯恩茅斯评分和染色体结果的预后价值。全组患者的中位生存期为22个月(RA 61.7个月,RARS 31.6个月,CMML 15.7个月,RAEB 10.3个月,RAEBt 8.2个月)。70例患者中有37例(52%)发现染色体异常。只有复杂核型患者与正常核型患者的生存差异有统计学意义(p = 0.02)。远离内皮表面的小母细胞(未成熟母细胞或ALIP的异常定位)的存在似乎是预测生存时间和向ANLL进展的主要预后因素,特别是在FAB亚组RA和RARS中。22例ALIP-患者合并RA/RARS的中位生存期为65个月,而ALIP+患者的中位生存期为31个月(p = 0.0006)。9例ALIP+患者(53%)发生ANLL,而3例ALIP-患者(13%)发生ANLL (p = 0.008)。通过重新定义ALIP并评估伴随细胞的数量和特征,区分组织学亚型与FAB亚组的相关性很大。我们的研究结果证明了骨髓活检和量化骨髓染色体变化的复杂性对预后的重要性。它应该有助于评估目前为MDS患者寻找有效治疗方法的尝试。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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