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Emerging evidence suggests that patients with MGRS and MGNS could benefit from treatments used for myeloma, Waldenstr\u00f6m macroglobulinemia, or chronic lymphocytic leukemia, depending on the underlying pathology. However, the treatment of MGRS and MGNS is not standardized, and potentially effective therapies might not be reimbursed because these conditions do not formally meet the criteria for malignant processes. The present review aims at establishing standards for the evaluation and management of MGRS and MGNS, which can facilitate the diagnosis of and provide therapeutic options for treating practitioners and patients affected by these conditions. The careful design and execution of clinical trials for patients with MGRS and MGNS are positively encouraged.<\/jats:p>","DOI":"10.1002\/ajh.26155","type":"journal-article","created":{"date-parts":[[2021,3,12]],"date-time":"2021-03-12T02:41:42Z","timestamp":1615516902000},"page":"846-853","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":37,"title":["The evaluation and management of monoclonal gammopathy of renal significance and monoclonal gammopathy of neurological significance"],"prefix":"10.1002","volume":"96","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9490-7532","authenticated-orcid":false,"given":"Jorge J.","family":"Castillo","sequence":"first","affiliation":[{"name":"Dana\u2010Farber Cancer Institute Harvard Medical School  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6975-1086","authenticated-orcid":false,"given":"Natalie S.","family":"Callander","sequence":"additional","affiliation":[{"name":"Carbone Cancer Center University of Wisconsin  Madison Wisconsin"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0630-8458","authenticated-orcid":false,"given":"Muhamed","family":"Baljevic","sequence":"additional","affiliation":[{"name":"Fred &amp; Pamela Buffett Cancer Center  Omaha"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas W.","family":"Sborov","sequence":"additional","affiliation":[{"name":"Huntsman Cancer Institute University of Utah  Salt Lake City Utah"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5392-9284","authenticated-orcid":false,"given":"Shaji","family":"Kumar","sequence":"additional","affiliation":[{"name":"Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,3,25]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa01133202"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-03-0801"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-07-445304"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41581-018-0077-4"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0272-6386(03)00412-8"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0039-1688422"},{"issue":"7","key":"e_1_2_7_8_1","first-page":"425","article-title":"Transjugular kidney biopsy: enabling safe tissue diagnosis in high risk patients","volume":"13","author":"Levi IM","year":"2011","journal-title":"Isr Med Assoc J"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2012.237"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ki.5001990"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-05-495929"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2015.65.0044"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(15)01120-4"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1817249"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1607751"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clml.2015.12.007"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.37.2649"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-04-015784"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2005.05366.x"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1081\/CNV-120005903"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.10213"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1802917"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2018.78.6426"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1501548"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1815281"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1900574"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1513257"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2010.30.8791"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.4065\/84.8.685"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00319857"},{"issue":"3","key":"e_1_2_7_31_1","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1111\/j.1529-8027.2010.00278.x","article-title":"European Federation of Neurological Societies\/Peripheral Nerve Society Guideline on management of paraproteinemic demyelinating neuropathies. 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