Validation of the new comprehensive cytogenetic scoring system (NCCSS) on 630 consecutive de novo MDS patients from a single institution.
This study evaluated whether the NCCSS truly improves the prognostic stratification of 630 consecutive de novo MDS patients and established which cytogenetic grouping [NCCSS or International Prognostic Scoring System (IPSS)], when combined with the WHO classification, best predicted the clinical outcome of myelodysplastic syndromes (MDS). The frequency of chromosomal defects was 53.8%. Clinical parameters, including number of cytopenias, WHO classification, IPSS cytogenetic categories and scores, NCCSS were all relevant for overall survival (OS) and leukemia-free survival (LFS) and were included in six distinct multivariate models compared by the Akaike Information Criterion (AIC). The most effective model to predict OS included the number of cytopenias, the WHO classification and the NCCSS, whereas the model including the number of cytopenias, blast cell percentage and the NCCSS and the model including the number of cytopenias the WHO classification and the NCCSS were almost equally effective to predict LFS. In conclusion, the NCCS (i) improves the prognostic stratification of the good and poor IPSS cytogenetic categories by introducing the very good and the very poor categories; (ii) is still incomplete in establishing the prognostic relevance of rare/double defects, (ii) applied to patients who receive supportive treatment only identifies five different prognostic subgroups, but applied to patients treated with specific therapies reveals only a trend toward a significantly different OS and LFS when patients of the poor and intermediate cytogenetic categories are compared, (iii) combined with the WHO classification is much more effective than the IPSS in predicting MDS clinical outcome.
Division of Hematology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy. p.bernasconi@smatteo.pv.it
Paolo
P
Bernasconi
Catherine
C
Klersy
Marina
M
Boni
Paola Maria
PM
Cavigliano
Irene
I
Dambruoso
Rita
R
Zappatore
D000368:Aged; D002869:Chromosome Aberrations; D015331:Cohort Studies; D020732:Cytogenetic Analysis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007558:Italy; D015470:Leukemia, Myeloid, Acute / Q000209:etiology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009190:Myelodysplastic Syndromes / Q000175:diagnosis* / Q000235:genetics* / Q000503:physiopathology / Q000628:therapy; D009503:Neutropenia / Q000209:etiology; D017410:Practice Guidelines as Topic; D011379:Prognosis; D016019:Survival Analysis; D013921:Thrombocytopenia / Q000209:etiology; D014944:World Health Organization