We have recently developed a risk model specific for patients with lower risk MDS that allows the identification of poor prognosis patients (

Leukemia
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2008
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22
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538
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). We showed that the majority of patients classified by IPSS as low risk actually have poor outcomes with respect to overall survival. The cause of death (COD) of these patients is not well understood. In order to guide early therapeutic intervention, it is important to identify whether the mortality in this group is attributable to causes related to MDS or whether death occurs due to age-related comorbidities. The aim of the present study was to examine the rate of disease-related deaths in a cohort of 269 deceased patients with low or intermediate-1 disease by IPSS at presentation to a tertiary care center from 1980 to 2006. Autopsy, final progress note, physician letter, or death certificate were used to determine COD. For each patient, we categorized the COD as disease-related vs. non-related. MDS-related death was defined as infection, bleeding, transformation to acute myeloid leukemia (AML), or disease progression. Non-related causes included accidents, coronary events, heart failure, respiratory and renal failure, non-hemorrhagic stroke, and chronic hepatitis. Median age at presentation and at time of death was 66 years (range 19–88) and 67 years (range 21–90), respectively. Overall median survival was 59 weeks (range = 1–831). By FAB classification, all subgroups were identified (RA = 41.3%, RARS = 5.8%, RAEB = 28.6%, RAEB-t = 12.3%, CMML = 12.0%). By IPSS score, most patients were Intermediate-1 (Int-1 = 79% and Low = 21%). Most patients had diploid cytogenetics (58.3%), and the most common cytogenetic abnormality was deletion of chromosome 5 (9.1%). Almost all patients received supportive care only. The COD was identified as MDS-related in 229 of 269 patients (85%). The most common disease-related COD was infection (39% of all deaths) followed by hemorrhage (34%) and transformation to AML (23%). The most frequent non-disease-related COD were cardiovascular events (19 of 40 patients). 8 of 269 patients died of a secondary neoplasm. Our data demonstrates that the majority of patients with low- or intermediate risk MDS will succumb to causes related to their underlying disease. Although these results need to be validated prospectively, early therapeutic intervention could be considered in the management of these patients to improve survival.

Topics:
myelodysplastic syndrome, risk reduction, prostatic hypertrophy risk score, hemorrhage, infections, refractory anemia with excess blasts, therapeutic intervention, cardiovascular event, cerebrovascular accident, disease progression

Disclosures: No relevant conflicts of interest to declare.

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2008, The American Society of Hematology
2008
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