{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T03:48:17Z","timestamp":1782532097595,"version":"3.54.5"},"reference-count":15,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2019,7,5]],"date-time":"2019-07-05T00:00:00Z","timestamp":1562284800000},"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":["Br J Haematol"],"published-print":{"date-parts":[[2019,11]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>IgM monoclonal gammopathies of undetermined significance (IgM <jats:styled-content style=\"fixed-case\">MGUS<\/jats:styled-content>) are associated with a risk of progression to Waldenstr\u00f6m macroglobulinaemia (<jats:styled-content style=\"fixed-case\">WM<\/jats:styled-content>) or other lymphoproliferative disorders (<jats:styled-content style=\"fixed-case\">LPD<\/jats:styled-content>) of 1\u20132% per year. We analysed 176 consecutive patients with IgM <jats:styled-content style=\"fixed-case\">MGUS<\/jats:styled-content> to evaluate risk factors for progression. With a median follow\u2010up of 83\u00a0months (1214 person\u2010years), 15 patients (8\u00b75%) progressed to <jats:styled-content style=\"fixed-case\">WM<\/jats:styled-content> (<jats:italic>n<\/jats:italic>\u00a0=\u00a014) or marginal zone lymphoma (<jats:italic>n<\/jats:italic>\u00a0=\u00a01). The rate of progression was 1\u00b732% per year (95% confidence interval [<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>] 0\u00b780\u20132\u00b720). The serum monoclonal protein concentration and the <jats:italic><jats:styled-content style=\"fixed-case\">MYD<\/jats:styled-content>88<\/jats:italic> mutation were independent risk factors for progression (Hazard ratio [<jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>] 23\u00b73, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 2\u00b70\u2013273\u00b73, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7012 and <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content> 24\u00b74, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 2\u00b72\u2013275\u00b73, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7010, respectively). The cumulative incidence of progression, while considering death as a competing event, was 11\u00b76% at 5\u00a0years and 38\u00b70% at 10\u00a0years in <jats:italic><jats:styled-content style=\"fixed-case\">MYD<\/jats:styled-content>88<\/jats:italic>\u2010mutated patients with a serum monoclonal protein of 10\u00a0g\/l or higher, as compared with 0% at 5\u00a0years and 1\u00b71% at 10\u00a0years for patients with none or one risk factor. This risk\u2010stratification model is able to identify a subset of patients with IgM <jats:styled-content style=\"fixed-case\">MGUS<\/jats:styled-content> at high risk of progression to <jats:styled-content style=\"fixed-case\">WM<\/jats:styled-content> or <jats:styled-content style=\"fixed-case\">LPD<\/jats:styled-content> who deserve a lifelong follow\u2010up.<\/jats:p>","DOI":"10.1111\/bjh.16086","type":"journal-article","created":{"date-parts":[[2019,7,5]],"date-time":"2019-07-05T13:59:26Z","timestamp":1562335166000},"page":"441-446","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["A risk\u2010stratification model based on the initial concentration of the serum monoclonal protein and <i><scp>MYD<\/scp>88<\/i> mutation status identifies a subset of patients with IgM monoclonal gammopathy of undetermined significance at high risk of progression to Waldenstr\u00f6m macroglobulinaemia or other lymphoproliferative disorders"],"prefix":"10.1111","volume":"187","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7304-1629","authenticated-orcid":false,"given":"Marzia","family":"Varettoni","sequence":"first","affiliation":[{"name":"Division of Haematology Fondazione IRCCS Policlinico San Matteo Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Zibellini","sequence":"additional","affiliation":[{"name":"Division of Haematology Fondazione IRCCS Policlinico San Matteo Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Boveri","sequence":"additional","affiliation":[{"name":"Unit of Anatomic Pathology Fondazione IRCCS Policlinico San Matteo Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Klersy","sequence":"additional","affiliation":[{"name":"Service of Biometry and Clinical Epidemiology Fondazione IRCCS Policlinico San Matteo Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Candido","sequence":"additional","affiliation":[{"name":"Division of Haematology Fondazione IRCCS Policlinico San Matteo Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2682-3414","authenticated-orcid":false,"given":"Sara","family":"Rattotti","sequence":"additional","affiliation":[{"name":"Division of Haematology Fondazione IRCCS Policlinico San Matteo Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virginia V.","family":"Ferretti","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine University of Pavia Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene","family":"Defrancesco","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine University of Pavia Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Mangiacavalli","sequence":"additional","affiliation":[{"name":"Division of Haematology Fondazione IRCCS Policlinico San Matteo Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria E.","family":"Nizzoli","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine University of Pavia Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Flospergher","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine University of Pavia Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caterina","family":"Zerbi","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine University of Pavia Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabio","family":"Bergamini","sequence":"additional","affiliation":[{"name":"Department 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