Abstract
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.
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