title: World Health Organization classification in combination with cytogenetic markers improves the prognostic stratification of patients with de novo primary myelodysplastic syndromes.
abstract: This study correlated chromosomal defects with French-American-British (FAB)/World Health Organization (WHO) classification subtypes, proposed a revised International Prognostic Scoring System (IPSS) cytogenetic grouping; and established which classification, when used with the IPSS cytogenetic categories, best predicted clinical outcome in the myelodysplastic syndromes (MDS). A higher prevalence of chromosomal defects and distinct defects were observed in patients with multi-lineage dysplasia and a blast cell percentage >10%. Abnormalities of the long arm of chromosome 3, del(7)(q31q35), trisomy 8, del(11)(q14q23), del(12p) and 20q- could be segregated from their respective IPSS cytogenetic categories and used to develop new cytogenetic subgroups. Clinical parameters, FAB/WHO classification, IPSS score and standard or revised cytogenetic categories were statistically relevant for overall survival (OS) and progression-free intervals (PFI) and were included within five distinct multivariate models compared by the Akaike Information Criterion. To predict OS, the best models included age, WHO classification and standard or revised IPSS cytogenetic categories; to predict PFI, the best model included the same variables and revised cytogenetic categories. In conclusion, (i) the WHO classification was associated with a more homogeneous cytogenetic pattern than the FAB classification, (ii) WHO classification and standard/revised IPSS cytogenetic categories were much more effective than IPSS for predicting MDS clinical outcome, (iii) revised cytogenetic subgroups predicted PFI more effectively than standard categories.
authors.affiliation: Division of Haematology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy. p.bernasconi@smatteo.pv.it
authors.forename: Paolo
authors.initials: P
authors.lastname: Bernasconi
authors.forename: Catherine
authors.initials: C
authors.lastname: Klersy
authors.forename: Marina
authors.initials: M
authors.lastname: Boni
authors.forename: Paola M
authors.initials: PM
authors.lastname: Cavigliano
authors.forename: Silvia
authors.initials: S
authors.lastname: Calatroni
authors.forename: Ilaria
authors.initials: I
authors.lastname: Giardini
authors.forename: Barbara
authors.initials: B
authors.lastname: Rocca
authors.forename: Rita
authors.initials: R
authors.lastname: Zappatore
authors.forename: Marilena
authors.initials: M
authors.lastname: Caresana
authors.forename: Irene
authors.initials: I
authors.lastname: Dambruoso
authors.forename: Mario
authors.initials: M
authors.lastname: Lazzarino
authors.forename: Carlo
authors.initials: C
authors.lastname: Bernasconi
mesh_terms: D000328:Adult; D000368:Aged; D002869:Chromosome Aberrations; D002872:Chromosome Deletion; D002877:Chromosomes, Human / Q000235:genetics; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D002881:Chromosomes, Human, Pair 12 / Q000235:genetics; D002890:Chromosomes, Human, Pair 20 / Q000235:genetics; D002893:Chromosomes, Human, Pair 3 / Q000235:genetics; D002897:Chromosomes, Human, Pair 7 / Q000235:genetics; D002898:Chromosomes, Human, Pair 8 / Q000235:genetics; D020732:Cytogenetic Analysis / Q000379:methods; D018450:Disease Progression; D005260:Female; D006801:Humans; D007621:Karyotyping / Q000379:methods; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000145:classification / Q000235:genetics* / Q000628:therapy; D011379:Prognosis; D014314:Trisomy / Q000235:genetics; D014944:World Health Organization