Prognostic Evaluation of Myelodysplastic Syndromes (MDS): Analysis of Deaths Due to Age-Related Causes.-猫眼课题宝

DOI：

10.1182/blood.v106.11.2543.2543

发表时间：

2005-11

期刊：

Blood

影响因子：

20.3

通讯作者：

中科院分区：

医学1区

文献类型：

作者：

F. Salvi;D. Gioia;S. Gatto;M. Bonferroni;G. Cametti;E. Campa;C. Ceretto;D. Cilloni;Antonella Darbesio;S. D’ardia;C. Dellacasa;D. Ferrero;F. Ficara;S. Franceschetti;R. Freilone;F. Marmont;E. Scassa;A. Tonso;M. Boccadoro;G. Gaidano;A. Gallamini;E. Gallo;M. Girotto;C. Marinone;G. Saglio;A. Levis

背景。不到 50% 的骨髓增生异常患者有明显的白血病进展。他们常常死于与年龄相关的问题，而这些问题与骨髓增生异常综合征（MDS）本身无关，并且很难为这组老年患者确定最佳治疗方法，其中积极策略的风险很高，而支持性护理可以构成最有用的选择。迄今为止缺乏对死亡原因的系统分析。工作的目的。分析一大群骨髓增生异常综合征的预后，特别是死亡原因。患者和方法。从 1999 年 1 月到 2005 年 6 月，783 例新发 MDS 病例的数据通过我们的网站前瞻性地记录到皮埃蒙特 MDS 登记册中。 32 例和 68 例分别因 RAEB-t 和 CMMoL 被排除。其余 680 例患者，即本分析的对象，可根据 WHO 分类细分如下： 99 例 RAEB-II； 160 RAEB-I； 104 RCMD； 317 除 RAEB 和 RCMD 之外的 MDS。有关共病和 IPSS 评分的数据分别适用于 457 名和 404 名患者。截至分析时，已记录 157 例死亡，并登记了 153 名患者的死因。结果。中位年龄为 73 岁（范围 27-95 岁），其中 151 名患者 (22%) 年龄超过 80 岁。399/457 名患者 (87%) 诊断时存在一种或多种合并症。 IPSS评分系统和WHO分类的预后作用均得到证实。死亡原因细分如下： 57 名患者（37%）因血细胞减少和/或白血病转化引起的并发症； 20 名患者感染（13%）；其余 76 名患者 (50%) 存在其他年龄或合并症相关原因。不同性别的死亡原因没有显着差异，而无关原因导致的死亡随着年龄的增加而增加，从 60 岁以下的 29% 上升到 80 岁以上的 61%（线性趋势检验：p=0.02）。在没有合并症的患者中，因血细胞减少和/或白血病转化和/或感染导致的死亡更为常见（75%），而根据合并症的数量，没有发现差异：患有一种、两种和三种相关疾病的患者分别为 44%、39% 和 55%。诊断亚组与无关原因死亡之间存在明显的显着关系：RAEB-II 为 21%； RAEB-I 为 51%； 53% 为 RCMD；除 RAEB 和 RCMD 外，MDS 为 76% (p<0.01)。 IPSS 评分与不相关原因之间存在明显的类似关系：分数 int-2/高为 27%，分数低/int-1 为 60% (p=0.01)。结论。对这组 MDS 患者的预后分析（关注死亡原因）表明，大多数患者死于无关的原因。年龄和合并症在确定该组患者的治疗策略时应发挥重要作用。因此，抗白血病治疗应仅限于一小部分诊断为 RAEB 且 IPSS 评分较高的患者。支持治疗的改进应该对大多数患者有用。

Background . A leukaemic evolution is evident in less than 50% of myelodysplastic patients. They can often die of age-related problems which are independent of myelodysplastic syndrome (MDS) itself, and the best therapy is difficult to define for this group of old patients, in which aggressive strategies are at high risk and supportive care can constitute the most useful option. A systematic analysis of causes of death is so far lacking. Aim of the work . To analyse the prognosis of a large group of myelodysplastic syndromes with particular reference to the causes of death. Patients and methods . From January 1999 to June 2005, data from 783 new cases of MDS were prospectively recorded into the Piedmont MDS register through our web site. Thirty two and 68 cases were excluded because RAEB-t and CMMoL respectively. The remaining 680 patients, who are the object of the present analysis, can be subdivided according to the WHO classification as follows: 99 RAEB-II; 160 RAEB-I; 104 RCMD; 317 MDS other than RAEB and RCMD. Data regarding co-morbidity and IPSS score are available for 457 and 404 patients respectively. At the moment of the analysis, 157 deaths were recorded and causes of death were registered for 153 patients. Results . Median age was 73 (range 27–95), with 151 patients (22%) older than 80. One or more co-morbidities were present at diagnosis in 399/457 (87%). The prognostic role of both IPSS scoring system and WHO classification were confirmed. The causes of death were subdivided as follows: complications due to cytopenia and/or leukaemic transformation in 57 patients (37%); infections in 20 patients (13%); other age or co-morbidity related causes in the remaining 76 patients (50%). No significant differences of causes of death were seen according to sex, while deaths from unrelated causes increased with increasing age from 29% under 60 years up to of 61% over 80 years (test for linear trend: p=0.02). Deaths due to cytopenia, and/or leukaemic transformations, and/or infections were more frequent in patients with no co-morbidities (75%), while no differences were seen according to the number of co-morbidities: 44%, 39% and 55% for patients with respectively one, two and three associated diseases. A significant relationship was evident between diagnostic subgroups and deaths from unrelated causes: 21% for RAEB-II; 51% for RAEB-I; 53% for RCMD; 76% for MDS other than RAEB and RCMD (p<0.01). A similar relationship was evident between IPSS score and causes from unrelated causes: 27% for score int-2/high and 60% for score low/int-1 (p=0.01). Conclusions . The prognostic analysis of this group of MDS patients with attention to the causes of dearth suggest that the majority of patients die of unrelated causes. Age and co-morbidities should play a major role in defining the treatment strategy of this group of patients. Anti-leukaemic treatments should therefore be limited to a small group of patients with diagnosis of RAEB and high IPSS score. An improvement in supportive treatment should be useful for the majority of patients.

Prognostic Evaluation of Myelodysplastic Syndromes (MDS): Analysis of Deaths Due to Age-Related Causes.

文献摘要

被引文献

相似文献