In chronic lymphocytic leukaemia (CLL), caution is warranted regarding the clinical implications of immunoglobulin variable heavy chain region (IGHV) rearrangements with a ‘borderline’ (BL) percentage of mutations (i.e. 97–97·9% IGHV identity). We analysed the IGHV mutational status in 759 untreated CLL patients (cohort 1). BL-CLL (n = 36, 5%) showed a time to first treatment (TFT) similar to that of M-CLL (n = 338) and significantly longer than that of UM-CLL (n = 385), despite the enrichment in subset #2 cases. In fact, CLLs belonging to subset #2 (n = 15/759, 2%) were significantly more frequent among BL-CLLs (n = 5/36, 14%), with a brief TFT. TFT of BL-CLL remained comparable to that of M-CLL also considering the 327 CLL patients evaluated at diagnosis. These findings were then validated in an independent cohort 2 of 759 newly diagnosed CLL patients (BL-CLL: n = 11, 1·4%) and in all newly diagnosed patients from cohorts 1 and 2 (n = 1 086, 84% stage A; BL-CLL: n = 47, 4·3%). BL-CLL at diagnosis showed a biological profile comparable to that of M-CLL with a low frequency of unfavourable prognostic markers, except for a significant enrichment in subset #2. Our data suggest that the prognosis of BL-CLL is good and similar to that of M-CLL, with the exception of subset #2 cases.

Redefining the prognostic likelihood of chronic lymphocytic leukaemia patients with borderline percentage of immunoglobulin variable heavy chain region mutations / Raponi, S.; Ilari, C.; Della Starza, I.; Cappelli, L. V.; Cafforio, L.; Piciocchi, A.; Arena, V.; Mariglia, P.; Mauro, F. R.; Gentile, M.; Cutrona, G.; Moia, R.; Favini, C.; Morabito, F.; Rossi, D.; Gaidano, G.; Guarini, A.; Del Giudice, I.; Foa, R.. - In: BRITISH JOURNAL OF HAEMATOLOGY. - ISSN 0007-1048. - 189:5(2020), pp. 853-859. [10.1111/bjh.16434]

Redefining the prognostic likelihood of chronic lymphocytic leukaemia patients with borderline percentage of immunoglobulin variable heavy chain region mutations

Della Starza I.;Cappelli L. V.;Cafforio L.;Piciocchi A.;Arena V.;Mauro F. R.;Del Giudice I.;Foa R.
2020

Abstract

In chronic lymphocytic leukaemia (CLL), caution is warranted regarding the clinical implications of immunoglobulin variable heavy chain region (IGHV) rearrangements with a ‘borderline’ (BL) percentage of mutations (i.e. 97–97·9% IGHV identity). We analysed the IGHV mutational status in 759 untreated CLL patients (cohort 1). BL-CLL (n = 36, 5%) showed a time to first treatment (TFT) similar to that of M-CLL (n = 338) and significantly longer than that of UM-CLL (n = 385), despite the enrichment in subset #2 cases. In fact, CLLs belonging to subset #2 (n = 15/759, 2%) were significantly more frequent among BL-CLLs (n = 5/36, 14%), with a brief TFT. TFT of BL-CLL remained comparable to that of M-CLL also considering the 327 CLL patients evaluated at diagnosis. These findings were then validated in an independent cohort 2 of 759 newly diagnosed CLL patients (BL-CLL: n = 11, 1·4%) and in all newly diagnosed patients from cohorts 1 and 2 (n = 1 086, 84% stage A; BL-CLL: n = 47, 4·3%). BL-CLL at diagnosis showed a biological profile comparable to that of M-CLL with a low frequency of unfavourable prognostic markers, except for a significant enrichment in subset #2. Our data suggest that the prognosis of BL-CLL is good and similar to that of M-CLL, with the exception of subset #2 cases.
2020
chronic lymphocytic leukaemia; immunoglobulin variable heavy chain; prognosis; somatic hypermutation
01 Pubblicazione su rivista::01a Articolo in rivista
Redefining the prognostic likelihood of chronic lymphocytic leukaemia patients with borderline percentage of immunoglobulin variable heavy chain region mutations / Raponi, S.; Ilari, C.; Della Starza, I.; Cappelli, L. V.; Cafforio, L.; Piciocchi, A.; Arena, V.; Mariglia, P.; Mauro, F. R.; Gentile, M.; Cutrona, G.; Moia, R.; Favini, C.; Morabito, F.; Rossi, D.; Gaidano, G.; Guarini, A.; Del Giudice, I.; Foa, R.. - In: BRITISH JOURNAL OF HAEMATOLOGY. - ISSN 0007-1048. - 189:5(2020), pp. 853-859. [10.1111/bjh.16434]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1411175
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