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Clinical and molecular characteristics of lymphoplasmacytic lymphoma not associated with an IgM monoclonal protein: A multicentric study of the Rete Ematologica Lombarda (REL) network
Author(s) -
Varettoni Marzia,
Boveri Emanuela,
Zibellini Silvia,
Tedeschi Alessandra,
Candido Chiara,
Ferretti Virginia Valeria,
Rizzo Ettore,
Doni Elisa,
Merli Michele,
Farina Lucia,
Goldaniga Maria,
Gallì Anna,
Rattotti Sara,
Frustaci Anna Maria,
Deodato Marina,
Bandiera Laura,
Isimbaldi Giuseppe,
Uccella Silvia,
Cabras Antonello Domenico,
Gianelli Umberto,
Baldini Luca,
Paulli Marco,
Arcaini Luca
Publication year - 2019
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.25600
Subject(s) - lymphoplasmacytic lymphoma , waldenstrom macroglobulinemia , medicine , cxcr4 , gastroenterology , macroglobulinemia , immunoglobulin m , bone marrow , monoclonal , pathology , lymphoma , immunology , antibody , immunoglobulin g , monoclonal antibody , multiple myeloma , receptor , chemokine
Lymphoplasmacytic lymphoma (LPL) is usually associated with a serum IgM paraprotein, corresponding to Waldenström's Macroglobulinemia (WM). Cases presenting with IgG or IgA, or without a monoclonal protein are extremely rare. We analyzed clinical characteristics, frontline treatment, and the outcome of 45 patients with non‐IgM LPL, and compared them with a control group of WM patients. The median age was similar, with significantly higher prevalence of females in non‐IgM LPL, than in WM patients (60% vs 39%, P = .016). Patients with non‐IgM LPL more frequently presented with lymphadenopathies (53% vs 15%, P < .001), splenomegaly (22% vs 8%, P = .015) or extranodal involvement (20% vs 8%, P = .05). In non‐IgM LPL a serum monoclonal protein and bone marrow infiltration were less common than in WM patients (69% and 84% of cases respectively, P < .001 for both comparisons). The MYD88 (L265P) mutation was found in 8/19 patients using allele‐specific polymerase chain reaction. A CXCR4 mutation was found in 4/17 cases using Sanger. In 16 patients we performed targeted next‐generation sequencing of genes MYD88, CXCR4, ARID1‐A, KMT2D, NOTCH2, TP53, PRDM1, CD79B, TRAF3, MYBBP1A, TNFAIP3 . Seven patients (44%) had a MYD88 mutation (S219C in one), four (25%) a CXCR4 mutation, three (19%) a KMT2D mutation, one (6%) a TP53 mutation and one (6%) a TRAF3 mutation. With a median follow‐up of 55.7 months, 36 non‐IgM LPL patients (80%) were treated. Non‐IgM LPL patients received more frequently anthracycline‐containing regimens, as compared with WM patients, who mainly received alkylating‐based therapies. Five‐year overall survival (OS) was 84%, similar to that of WM patients.