Abstract:
:A risk score based on three biological features (CD38, ZAP-70, and IGHV mutational status) was previously developed to predict progression-free survival (PFS) in untreated Binet A CLL patients. Here we perform a score validation analysis in a prospective and independent cohort of patients. Biological markers (CD38, ZAP-70, and IGHV mutational status) and gene expression profiles (GEP) of leukemic cells from CLL patients included in a prospective multicenter observational study (O-CLL1-GISL protocol, clinicaltrial.gov ID:NCT00917549) were used to assess the value and reproducibility of this score. To date, 468 Binet A patients were classified as low- (0 positive marker), intermediate- (1 positive marker), or high-risk (2 or 3 positive markers) using the progression risk score. The 3-year PFS probability was 91.7%, 82.9%, and 57.4% for low-, intermediate-, and high-risk (P < 0.0001) cases, respectively. These values were similar to those found in the original cohort. At Cox multivariate analysis, Rai stage, absolute lymphocyte count, progression risk score, and β-2 microglobulin maintained an independent prognostic impact on PFS. This score remained a predictor of progression when analysis was limited to 371 Rai 0 cases (P < 0.0001). Finally, the cells from the different CLL risk groups showed differences in their gene expression patterns. These results confirm the ability of this progression risk score to predict PFS among Binet A patients. The utility of the score was also extended by demonstrating that it retains prognostic value when applied exclusively to Rai 0 patients. Specific transcriptional patterns were significantly associated with risk groups.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Gentile M,Cutrona G,Mosca L,Matis S,Fabris S,Lionetti M,Ilariucci F,Zupo S,Musolino C,Levato L,Molica S,Di Raimondo F,Vincelli I,Di Rienzo N,Pesce EA,Angrilli F,Federico M,Neri A,Ferrarini M,Morabito Fdoi
10.1002/ajh.23729subject
Has Abstractpub_date
2014-07-01 00:00:00pages
743-50issue
7eissn
0361-8609issn
1096-8652journal_volume
89pub_type
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