{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T07:51:13Z","timestamp":1790754673768,"version":"4.1.0"},"reference-count":29,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2019,9,9]],"date-time":"2019-09-09T00:00:00Z","timestamp":1567987200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2019,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Lymphoplasmacytic lymphoma (LPL) is usually associated with a serum IgM paraprotein, corresponding to Waldenstr\u00f6m'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\u2010IgM LPL, and compared them with a control group of WM patients. The median age was similar, with significantly higher prevalence of females in non\u2010IgM LPL, than in WM patients (60% vs 39%, <jats:italic>P<\/jats:italic> =\u2009.016). Patients with non\u2010IgM LPL more frequently presented with lymphadenopathies (53% vs 15%, <jats:italic>P<\/jats:italic> &lt;\u2009.001), splenomegaly (22% vs 8%, <jats:italic>P<\/jats:italic> =\u2009.015) or extranodal involvement (20% vs 8%, <jats:italic>P<\/jats:italic> =\u2009.05). In non\u2010IgM LPL a serum monoclonal protein and bone marrow infiltration were less common than in WM patients (69% and 84% of cases respectively, <jats:italic>P<\/jats:italic> &lt;\u2009.001 for both comparisons). The <jats:italic>MYD88<\/jats:italic> (L265P) mutation was found in 8\/19 patients using allele\u2010specific polymerase chain reaction. A <jats:italic>CXCR4<\/jats:italic> mutation was found in 4\/17 cases using Sanger. In 16 patients we performed targeted next\u2010generation sequencing of genes <jats:italic>MYD88, CXCR4, ARID1\u2010A, KMT2D, NOTCH2, TP53, PRDM1, CD79B, TRAF3, MYBBP1A, TNFAIP3<\/jats:italic>. Seven patients (44%) had a <jats:italic>MYD88<\/jats:italic> mutation (S219C in one), four (25%) a <jats:italic>CXCR4<\/jats:italic> mutation, three (19%) a <jats:italic>KMT2D<\/jats:italic> mutation, one (6%) a <jats:italic>TP53<\/jats:italic> mutation and one (6%) a <jats:italic>TRAF3<\/jats:italic> mutation. With a median follow\u2010up of 55.7 months, 36 non\u2010IgM LPL patients (80%) were treated. Non\u2010IgM LPL patients received more frequently anthracycline\u2010containing regimens, as compared with WM patients, who mainly received alkylating\u2010based therapies. Five\u2010year overall survival (OS) was 84%, similar to that of WM patients.<\/jats:p>","DOI":"10.1002\/ajh.25600","type":"journal-article","created":{"date-parts":[[2019,8,5]],"date-time":"2019-08-05T06:14:10Z","timestamp":1564985650000},"page":"1193-1199","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Clinical and molecular characteristics of lymphoplasmacytic lymphoma not associated with an IgM monoclonal protein: A multicentric study of the Rete Ematologica Lombarda (REL) network"],"prefix":"10.1002","volume":"94","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7304-1629","authenticated-orcid":false,"given":"Marzia","family":"Varettoni","sequence":"first","affiliation":[{"name":"Division of Hematology Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Boveri","sequence":"additional","affiliation":[{"name":"Anatomic Pathology Section Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Zibellini","sequence":"additional","affiliation":[{"name":"Division of Hematology Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Tedeschi","sequence":"additional","affiliation":[{"name":"Department of Hematology Niguarda Cancer Center, Niguarda Hospital  Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Candido","sequence":"additional","affiliation":[{"name":"Division of Hematology Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virginia Valeria","family":"Ferretti","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine University of Pavia  Pavia 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