PURPOSE: To investigate the incidence and clinical relevance of classic and new prognostic markers, IGHV gene mutational status, and chromosomal abnormalities in cMBLs compared to Rai stage 0 chronic lymphocytic leukemia (Rai0-CLL). EXPERIMENTAL DESIGN: A group of 136 clinical monoclonal B lymphocytosis (cMBL) patients and a group of 216 Rai0-CLL cases were investigated prospectively. RESULTS: IGHV mutated cases were significantly more frequent among cMBLs (P=0.005), while the distribution of CD38 and ZAP-70 positive cases, of patients with NOTCH1 and SF3B1 mutations or exhibiting the major CLL cytogenetic abnormalities was similar in the two groups. Moreover, no significant differences were found neither in IGHV/IGHD/IGHJ gene usage nor in the overall prevalence of stereotyped IGHV gene sequences. Cells from cMBL and Rai0-CLL exhibited similar gene and miRNA signatures; in addition, when grouped according to the IGHV mutational status, IGHV unmutated cases showed different transcriptional signatures compared to IGHV mutated patients, irrespectively of the cMBL or Rai0-CLL classification. cMBL diagnosis per se was predictive of longer progression free survival. CONCLUSIONS: Our study based on a prospective series of patients indicates that no major differences exist between the circulating cells from cMBL and Rai0-CLL, at least based on a comparison of the markers utilized in the study. This possibly suggests that the two conditions mainly differ in the initial size of the monoclonal cell population, which may influence the subsequent timing of clonal expansion and clinical manifestations.
Clinical Monoclonal B lymphocytosis versus Rai 0 Chronic Lymphocytic Leukemia: a Comparison of Cellular, Cytogenetic, Molecular, and Clinical Features
FERRACIN, MANUELA;
2013
Abstract
PURPOSE: To investigate the incidence and clinical relevance of classic and new prognostic markers, IGHV gene mutational status, and chromosomal abnormalities in cMBLs compared to Rai stage 0 chronic lymphocytic leukemia (Rai0-CLL). EXPERIMENTAL DESIGN: A group of 136 clinical monoclonal B lymphocytosis (cMBL) patients and a group of 216 Rai0-CLL cases were investigated prospectively. RESULTS: IGHV mutated cases were significantly more frequent among cMBLs (P=0.005), while the distribution of CD38 and ZAP-70 positive cases, of patients with NOTCH1 and SF3B1 mutations or exhibiting the major CLL cytogenetic abnormalities was similar in the two groups. Moreover, no significant differences were found neither in IGHV/IGHD/IGHJ gene usage nor in the overall prevalence of stereotyped IGHV gene sequences. Cells from cMBL and Rai0-CLL exhibited similar gene and miRNA signatures; in addition, when grouped according to the IGHV mutational status, IGHV unmutated cases showed different transcriptional signatures compared to IGHV mutated patients, irrespectively of the cMBL or Rai0-CLL classification. cMBL diagnosis per se was predictive of longer progression free survival. CONCLUSIONS: Our study based on a prospective series of patients indicates that no major differences exist between the circulating cells from cMBL and Rai0-CLL, at least based on a comparison of the markers utilized in the study. This possibly suggests that the two conditions mainly differ in the initial size of the monoclonal cell population, which may influence the subsequent timing of clonal expansion and clinical manifestations.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
