Prognostic features of patients with myelodysplastic syndromes aged < 50 years: update of a single-institution experience.
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.
Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy. breccia@bce.uniroma1.it
Massimo
M
Breccia
Paola
P
Finsinger
Giuseppina
G
Loglisci
Michelina
M
Santopietro
Adriano
A
Salaroli
Alessandra
A
Serrao
Roberto
R
Latagliata
Paola
P
Volpicelli
Luigi
L
Petrucci
Mauro
M
Nanni
Giuliana
G
Alimena
D000208:Acute Disease; D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D001554:Benzene / Q000506:poisoning; D002471:Cell Transformation, Neoplastic / Q000187:drug effects / Q000235:genetics; D002869:Chromosome Aberrations; D002897:Chromosomes, Human, Pair 7 / Q000235:genetics; D002898:Chromosomes, Human, Pair 8 / Q000235:genetics; D018450:Disease Progression; D005260:Female; D006801:Humans; D007938:Leukemia / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009006:Monosomy; D009190:Myelodysplastic Syndromes / Q000139:chemically induced / Q000235:genetics* / Q000473:pathology; D009784:Occupational Diseases / Q000139:chemically induced / Q000235:genetics* / Q000473:pathology; D016273:Occupational Exposure; D011379:Prognosis; D012997:Solvents / Q000506:poisoning; D016019:Survival Analysis; D014314:Trisomy; D055815:Young Adult
D012997:Solvents; D001554:Benzene