Clinical and biological characteristics of lymphoplasmacytic lymphoma not associated with an IgM monoclonal protein: a multicentric study of the Rete Ematologica Lombarda (REL) network

Lymphoplasmacytic lymphoma (LPL) is a low-grade B-cell neoplasm exhibiting a spectrum of B-cell differentiation ranging from small lymphocytes to plasma cells. In the majority of cases LPL is associated with a serum immunoglobulin M (IgM) paraprotein corresponding to the clinicopathologic entity of Waldenström’s Macroglobulinemia (WM). In a minority of cases of LPL the paraprotein is absent or is of IgG or IgA isotype. These cases are rare and their relationship with WM still unclear. Moreover, patients with LPL not associated with an IgM monoclonal protein are usually excluded from interventional studies focusing on WM. While the genomic landscape of WM patients has been studied in the recent years, revealing MYD88 and CXCR4 mutations as the most frequent somatic mutations, little is known about the molecular characteristics of non IgM LPL. The aim of this study was to evaluate the clinical and molecular characteristics, patterns of treatment and outcome of patients with LPL not associated with an IgM paraprotein (non IgM LPL) as compared with those of WM patients.

Caratteristiche cliniche e biologiche del linfoma linfoplasmacitico non associato ad una componente monoclonale IgM: uno studio multicentrico sulla Rete Ematologica Lombarda (REL)