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

Marzia Varettoni, Emanuela Boveri, Silvia Zibellini, Alessandra Tedeschi, Chiara Candido, Virginia Valeria Ferretti, Ettore Rizzo, Elisa Doni, Michele Merli, Lucia Farina, Maria Goldaniga, Anna Gallì, Sara Rattotti, Anna Maria Frustaci, Marina Deodato, Laura Bandiera, Giuseppe Isimbaldi, Silvia Uccella, Antonello Domenico Cabras, Umberto GianelliLuca Baldini, Marco Paulli, Luca Arcaini

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)1193-1199
JournalAmerican Journal of Hematology
Volume94
Issue number11
DOIs
Publication statusPublished - 2019

ASJC Scopus subject areas

  • Hematology

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