Monoclonal B-cell lymphocytosis: Does the elderly patient need a specialistic approach?

Abstract

Monoclonal B-cell lymphocytosis (MBL) is defined by the presence of a monoclonal B-cell population in the peripheral blood (PB) at a concentration of <5 × 10⁹/l and no signs or symptoms of a lymphoproliferative disorder. In around 75% of cases, the immunophenotype of the clonal B-cell expansions is superimposable to that of chronic lymphocytic leukemia (CLL), thus defined "CLL-like". Other cases may coexpress CD19, CD5, bright CD20, and lack CD23 ("atypical CLL"), while others are CD5-negative ("non-CLL"). Beside the immunophenotypic profile, a key distinction is based on the B-cell count, stratifying the MBL category in low (<0.5 × 10⁹/l) or high-count (≥0.5 × 10⁹/l). Low-count (LC) MBL is recognized in general population studies and it is not associated with lymphocytosis. High-count (HC) MBL is identified during the clinical work-up for lymphocytosis and carries a risk of progression to CLL requiring therapy of 1-2% per year in most series, warranting clinical monitoring over time. At the time of MBL diagnosis, the key point is the careful evaluation and exclusion of differential diagnoses. After the initial workup, the follow-up at a referral center by a hematologist would be desirable as, in addition to the obvious risk of progression to clinically relevant CLL, the appropriate management of MBL individuals should take into account the risk of developing infections, other cancers and autoimmune disorders. For those cases who indeed bear a risk, though limited, of clinical consequences, such as the majority of HC-MBL cases, current evidences suggest that they may benefit from a tailored and specialized approach.

Keywords: CLL; MBL; Monoclonal B-cell lymphocytosis; chronic lymphocytic leukemia; high-count; low-count.