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Prognostic features of patients with myelodysplastic syndromes aged < 50 years: update of a single-institution experience.

Abstract
Fewer than 10% of patients with myelodysplastic syndromes (MDS) are younger than 50 years. A series of 91 younger patients (median age 44 years with female prevalence) are reported and compared with elderly patients. Frequent karyotypic changes were trisomy 8 (9.8%) and monosomy 7 (5%). Twenty-three patients had occupational exposure to potential mutagens (benzene and solvents), with a male predominance, higher frequency of refractory cytopenia with multilineage dysplasia (RCMD) (52%) and higher frequency of monosomy 7 (21.7%). At a median follow-up of 72 months, 22 patients (24%) evolved to acute leukemia, with higher frequency being observed among the exposed cohort (39% vs. 19% non-exposed). Unfavorable factors for overall survival were: age > 40 years, > 5% of blasts, trilinear bone marrow involvement and intermediate-high World Health Organization Prognostic Scoring System (WPSS) risk. The present results suggest that younger MDS could be identified as a distinct subset. For patients belonging to the low/intermediate-I risk group, due to a low transformation rate, aggressive approaches should rarely be recommended.
AuthorsMassimo Breccia, Paola Finsinger, Giuseppina Loglisci, Michelina Santopietro, Adriano Salaroli, Alessandra Serrao, Roberto Latagliata, Paola Volpicelli, Luigi Petrucci, Mauro Nanni, Giuliana Alimena
JournalLeukemia & lymphoma (Leuk Lymphoma) Vol. 53 Issue 12 Pg. 2439-43 (Dec 2012) ISSN: 1029-2403 [Electronic] United States
PMID22647078 (Publication Type: Journal Article)
Chemical References
  • Solvents
  • Benzene
Topics
  • Acute Disease
  • Adult
  • Aged
  • Analysis of Variance
  • Benzene (poisoning)
  • Cell Transformation, Neoplastic (drug effects, genetics)
  • Chromosome Aberrations
  • Chromosomes, Human, Pair 7 (genetics)
  • Chromosomes, Human, Pair 8 (genetics)
  • Disease Progression
  • Female
  • Humans
  • Leukemia (genetics, pathology)
  • Male
  • Middle Aged
  • Monosomy
  • Myelodysplastic Syndromes (chemically induced, genetics, pathology)
  • Occupational Diseases (chemically induced, genetics, pathology)
  • Occupational Exposure
  • Prognosis
  • Solvents (poisoning)
  • Survival Analysis
  • Trisomy
  • Young Adult

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