B-cell receptor, clinical course and prognosis in chronic lymphocytic leukaemia: the growing saga of the IGHV3 subgroup gene usage

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BlankB-cell receptor, clinical course and prognosis in chronic lymphocytic

leukaemia: the growing saga of the IGHV3 subgroup gene usage.

M Dal-Bo, I Del Giudice, R Bomben, D Capello, F Bertoni, F Forconi, L ti,

D Rossi, A Zucchetto, G Pozzato, R Marasca, DG Efremov, A Guarini, G Del Poeta,

R Foa, G Gaidano, and V Gattei

Br J Haematol, February 8, 2011;

Clinical and Experimental Onco-Haematology Unit, Centro di Riferimento

Oncologico, I.R.C.C.S., Aviano (PN) Division of Haematology, Department of

Cellular Biotechnologies and Haematology, Sapienza University, Rome Division of

Haematology, Department of Clinical and Experimental Medicine & IRCAD, Amedeo

Avogadro University of Eastern Piedmont, Novara, Italy Laboratory of

Experimental Oncology and Lymphoma Unit, Oncology Institute of Southern

Switzerland (IOSI), Bellinzona, Switzerland Division of Haematology and

Transplant, Department of Clinical Medicine and Immunological Sciences,

University of Siena, Siena Haematology Institute, Catholic University " Sacro

Cuore " , Rome Department of Internal Medicine and Haematology, Maggiore General

Hospital, University of Trieste, Trieste Division of Haematology - Department of

Oncology and Haematology-University of Modena and Reggio Emilia, Modena

Molecular Haematology, ICGEB Outstation-Monterotondo, Rome Division of

Haematology, S.Eugenio Hospital and University of Tor Vergata, Rome, Italy.

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.

PMID: 21303354 .