Maschek H, Gutzmer R, Choritz H, Georgii A
Pathologisches Institut, Medizinische Hochschule Hannover, Germany.
Ann Hematol. 1995 Jun;70(6):301-8. doi: 10.1007/BF01696616.
To compare the prognostic value of scoring systems in primary myelodysplastic syndromes (pMDS), four clinicohematological systems (Rennes, Bournemouth, Düsseldorf, Pavia) and the histopathological Hannover Scoring System were applied to 415 MDS patients from the Bone Marrow Registry of Hannover Medical School. According to the FAB classification, 180 patients (43%) were diagnosed as RA, 33 (8%) as RARS, 99 (24%) as RAEB, 36 (9%) as RAEBt, and 48 (12%) as CMMOL; 19 patients (4%) were not further classified (MDS.UC). All scoring systems revealed three or four groups of patients with significantly different survival times. The ranges and standard deviations in these groups were similar but high in all scoring systems. A good differentiation between short-term survivors (< 1 year survival time) and intermediate-term survivors (1-4 years) was possible with all tested scoring systems, but the differentiation between intermediate- and long-term survivors (> 4 years) was not distinctive enough. The problem of risk assessment in the single patient is furthermore elucidated by the values of specificity and sensitivity, which were relatively low in all scoring systems tested. Best results were yielded by the Bournemouth Score for long-term survivors and the Düsseldorf and Hannover Score for intermediate- and short-term survivors. Multivariate analysis of all parameters used in the scoring systems showed the highest negative impact on survival for increase of myeloblasts, anemia, myelofibrosis, high age of the patient, and abnormal localization of immature precursors (ALIPs). Therefore, the histopathological Hannover Scoring System is not only equal to clinicohematological risk assessment in pMDS, but also includes important independent prognostic parameters. Risk assessment for the individual low-risk MDS patient using only initial parameters may be rendered impossible due to the biological nature of pMDS. Therefore, sequential analysis is needed to elucidate random events which alter the prognosis.
为比较各评分系统对原发性骨髓增生异常综合征（pMDS）的预后评估价值，将四种临床血液学评分系统（雷恩、伯恩茅斯、杜塞尔多夫、帕维亚）及组织病理学汉诺威评分系统应用于汉诺威医学院骨髓登记处的415例MDS患者。根据FAB分类，180例患者（43%）诊断为RA，33例（8%）为RARS，99例（24%）为RAEB，36例（9%）为RAEBt，48例（12%）为CMMOL；19例患者（4%）未进一步分类（MDS.UC）。所有评分系统均显示出三组或四组患者的生存时间有显著差异。这些组别的范围和标准差相似，但在所有评分系统中均较高。所有测试的评分系统都能较好地区分短期存活者（生存时间<1年）和中期存活者（1 - 4年），但中期和长期存活者（>4年）之间的区分不够明显。此外，所有测试评分系统的特异性和敏感性值相对较低，这进一步说明了单例患者风险评估的问题。对于长期存活者，伯恩茅斯评分效果最佳；对于中期和短期存活者，杜塞尔多夫评分和汉诺威评分效果最佳。对评分系统中使用的所有参数进行多因素分析显示，原始粒细胞增多、贫血、骨髓纤维化、患者高龄以及幼稚前体细胞异常定位（ALIPs）对生存的负面影响最大。因此，组织病理学汉诺威评分系统不仅与pMDS的临床血液学风险评估相当，还包含重要的独立预后参数。由于pMDS的生物学特性，仅使用初始参数对个体低风险MDS患者进行风险评估可能无法实现。因此，需要进行序贯分析以阐明改变预后的随机事件。
