2011•British Journal of HaematologyOpen access
B‐cell receptor, clinical course and prognosis in chronic lymphocytic leukaemia: the growing saga of the IGHV3 subgroup gene usage
Michele Dal Bo, Ilaria Del Giudice, Riccardo Bomben, Daniela Capello, Francesco Bertoni, Francesco Forconi, Luca Laurenti, Davide Rossi, Antonella Zucchetto, Gabriele Pozzato, Roberto Marasca, Dimitar G. Efremov, Anna Guarini, Giovanni Del Poeta, Robin Foà, Gianluca Gaïdano, Valter Gattei
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Abstract
The immunoglobulin heavy chain variable gene (IGHV) mutational status has been recognized as an important predictor of prognosis in chronic lymphocytic leukaemia (CLL) since 1999. More recently, other features of the B-cell receptor, such as stereotypy, have been identified as capable of refining the prognostic potential of IGHV status in the clinical assessment of CLL patients. In this context, different genes belonging to the IGHV3 subgroup, the most frequently used subgroup in CLL, have been shown to denote disease subsets that either display a bad prognosis (i.e. IGHV3-21, IGHV3-23) or are associated with particularly good clinical outcomes, including a highly stable/indolent clinical course, even prone to spontaneous regression (i.e. IGHV3-72, IGHV3-30). The present review focuses on the molecular and biological features of CLL-expressing specific genes belonging to the IGHV3 subgroup that are known to mark disease subsets with completely different clinical courses, and may be possibly related to CLL pathogenesis via antigen and/or superantigen involvement.