{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T05:38:54Z","timestamp":1774330734961,"version":"3.50.1"},"reference-count":73,"publisher":"MDPI AG","issue":"20","license":[{"start":{"date-parts":[[2021,10,13]],"date-time":"2021-10-13T00:00:00Z","timestamp":1634083200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["PI19\/00669"],"award-info":[{"award-number":["PI19\/00669"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Monoclonal gammopathy of undetermined significance (MGUS) is defined as the presence of a monoclonal protein (M-protein) produced by a small amount of plasma cells. The majority of patients remain asymptomatic; however, a fraction of them develop clinical manifestations related to the monoclonal gammopathy despite not fulfilling criteria of multiple myeloma or other lymphoproliferative disorder. These patients constitute an emerging clinical issue coined as monoclonal gammopathy of clinical significance (MGCS). The mechanisms involved are poorly understood, and literature is scarce regarding management. The clinical spectrum involves symptoms related to renal, neurologic, skin, ocular, or bleeding manifestations, requiring a multidisciplinary approach. Treatment strategies rely on the basis of symptomatic disease and the M-protein isotype. In this review, we focus on MGCS other than renal, as the latter was earliest recognized and better known. We review the literature and discuss management from diagnosis to treatment based on illustrative cases from daily practice.<\/jats:p>","DOI":"10.3390\/cancers13205131","type":"journal-article","created":{"date-parts":[[2021,10,13]],"date-time":"2021-10-13T09:33:02Z","timestamp":1634117582000},"page":"5131","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Treatment of Patients with Monoclonal Gammopathy of Clinical Significance"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1752-3081","authenticated-orcid":false,"given":"David F.","family":"Moreno","sequence":"first","affiliation":[{"name":"Amyloidosis and Multiple Myeloma Unit, Hospital Cl\u00ednic, 08036 Barcelona, Spain"},{"name":"Institut d\u2019Investigacions Biom\u00e8diques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laura","family":"Rosi\u00f1ol","sequence":"additional","affiliation":[{"name":"Amyloidosis and Multiple Myeloma Unit, Hospital Cl\u00ednic, 08036 Barcelona, Spain"},{"name":"Institut d\u2019Investigacions Biom\u00e8diques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda Teresa","family":"Cibeira","sequence":"additional","affiliation":[{"name":"Amyloidosis and Multiple Myeloma Unit, Hospital Cl\u00ednic, 08036 Barcelona, Spain"},{"name":"Institut d\u2019Investigacions Biom\u00e8diques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joan","family":"Blad\u00e9","sequence":"additional","affiliation":[{"name":"Amyloidosis and Multiple Myeloma Unit, Hospital Cl\u00ednic, 08036 Barcelona, Spain"},{"name":"Institut d\u2019Investigacions Biom\u00e8diques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4930-9255","authenticated-orcid":false,"given":"Carlos","family":"Fern\u00e1ndez de Larrea","sequence":"additional","affiliation":[{"name":"Amyloidosis and Multiple Myeloma Unit, Hospital Cl\u00ednic, 08036 Barcelona, Spain"},{"name":"Institut d\u2019Investigacions Biom\u00e8diques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,10,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2765","DOI":"10.1056\/NEJMcp052790","article-title":"Monoclonal Gammopathy of Undetermined Significance","volume":"355","year":"2006","journal-title":"N. Engl. J. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"e538","DOI":"10.1016\/S1470-2045(14)70442-5","article-title":"International Myeloma Working Group Updated Criteria for the Diagnosis of Multiple Myeloma","volume":"15","author":"Rajkumar","year":"2014","journal-title":"Lancet Oncol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1362","DOI":"10.1056\/NEJMoa054494","article-title":"Prevalence of Monoclonal Gammopathy of Undetermined Significance","volume":"354","author":"Kyle","year":"2006","journal-title":"N. Engl. J. Med."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1056\/NEJMoa1709974","article-title":"Long-Term Follow-up of Monoclonal Gammopathy of Undetermined Significance","volume":"378","author":"Kyle","year":"2018","journal-title":"N. Engl. J. Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1053\/sonc.2003.50082","article-title":"Clinicopathological Definition of Waldenstrom\u2019s Macroglobulinemia: Consensus Panel Recommendations from the Second International Workshop on Waldenstrom\u2019s Macroglobulinemia","volume":"30","author":"Owen","year":"2003","journal-title":"Semin. Oncol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"266","DOI":"10.1002\/ajh.25292","article-title":"Waldenstr\u00f6m Macroglobulinemia: 2019 Update on Diagnosis, Risk Stratification, and Management","volume":"94","author":"Gertz","year":"2019","journal-title":"Am. J. Hematol."},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Moreno, D.F., Pereira, A., Tovar, N., Cibeira, M.T., Magnano, L., Rozman, M., L\u00f3pez-Guerra, M., Colomer, D., Mart\u00edn-Antonio, B., and Jim\u00e9nez-Segura, R. (2021). Defining an Ultra-Low Risk Group in Asymptomatic IgM Monoclonal Gammopathy. Cancers, 13.","DOI":"10.3390\/cancers13092055"},{"key":"ref_8","doi-asserted-by":"crossref","unstructured":"Lomas, O.C., Mouhieddine, T.H., Tahri, S., and Ghobrial, I.M. (2020). Monoclonal Gammopathy of Undetermined Significance (MGUS)\u2014Not So Asymptomatic after All. Cancers, 12.","DOI":"10.3390\/cancers12061554"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"2520","DOI":"10.1182\/blood-2006-03-001164","article-title":"Dangerous Small B-Cell Clones","volume":"108","author":"Merlini","year":"2006","journal-title":"Blood"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1478","DOI":"10.1182\/blood-2018-04-839480","article-title":"Monoclonal Gammopathy of Clinical Significance: A Novel Concept with Therapeutic Implications","volume":"132","author":"Fermand","year":"2018","journal-title":"Blood"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"4292","DOI":"10.1182\/blood-2012-07-445304","article-title":"Monoclonal Gammopathy of Renal Significance: When MGUS Is No Longer Undetermined or Insignificant","volume":"120","author":"Leung","year":"2012","journal-title":"Blood"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1038\/s41581-018-0077-4","article-title":"The Evaluation of Monoclonal Gammopathy of Renal Significance: A Consensus Report of the International Kidney and Monoclonal Gammopathy Research Group","volume":"15","author":"Leung","year":"2019","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"3583","DOI":"10.1182\/blood-2013-05-495929","article-title":"How I Treat Monoclonal Gammopathy of Renal Significance (MGRS)","volume":"122","author":"Fermand","year":"2013","journal-title":"Blood"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1464","DOI":"10.1182\/blood-2018-07-865642","article-title":"M-Protein\u2013Related Disorders: MGCS","volume":"132","author":"Cibeira","year":"2018","journal-title":"Blood"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1748","DOI":"10.1016\/j.ophtha.2015.05.029","article-title":"Paraproteinemic Keratopathy","volume":"122","author":"Milman","year":"2015","journal-title":"Ophthalmology"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1111\/bjh.13196","article-title":"Clinico-Biological Characteristics and Treatment of Type I Monoclonal Cryoglobulinaemia: A Study of 64 Cases","volume":"168","author":"Harel","year":"2015","journal-title":"Br. J. Haematol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1111\/his.12768","article-title":"Crystal-Storing Histiocytosis: A Clinicopathological Study of 13 Cases","volume":"68","author":"Miranda","year":"2016","journal-title":"Histopathology"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"2620","DOI":"10.1182\/blood.2020006913","article-title":"Management of AL Amyloidosis in 2020","volume":"136","author":"Palladini","year":"2020","journal-title":"Blood"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"576","DOI":"10.1182\/blood-2018-09-872028","article-title":"Randall-Type Monoclonal Immunoglobulin Deposition Disease: Novel Insights from a Nationwide Cohort Study","volume":"133","author":"Joly","year":"2019","journal-title":"Blood"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1968","DOI":"10.1093\/brain\/124.10.1968","article-title":"The Clinical and Laboratory Features of Chronic Sensory Ataxic Neuropathy with Anti-Disialosyl IgM Antibodies","volume":"124","author":"Willison","year":"2001","journal-title":"Brain"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1111\/bjh.14492","article-title":"Investigation and Management of IgM and Waldenstr\u00f6m-Associated Peripheral Neuropathies: Recommendations from the IWWM-8 Consensus Panel","volume":"176","author":"Kersten","year":"2017","journal-title":"Br. J. Haematol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"E3689","DOI":"10.1073\/pnas.1619386114","article-title":"Selective in Vivo Removal of Pathogenic Anti-MAG Autoantibodies, an Antigen-Specific Treatment Option for Anti-MAG Neuropathy","volume":"114","author":"Herrendorff","year":"2017","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1007\/s00277-016-2650-x","article-title":"Distinct Mechanisms Account for Acquired von Willebrand Syndrome in Plasma Cell Dyscrasias","volume":"95","author":"Dicke","year":"2016","journal-title":"Ann. Hematol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1159\/000345418","article-title":"Specific Binding of Paraprotein to Platelet Receptors as a Cause of Platelet Dysfunction in Monoclonal Gammopathies","volume":"130","author":"Djunic","year":"2013","journal-title":"Acta Haematol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1016\/j.jaad.2015.07.031","article-title":"The Spectrum of Neutrophilic Dermatoses Associated with Monoclonal Gammopathy: Association with IgA Isotype and Inflammatory Profile","volume":"73","author":"Szalat","year":"2015","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"974","DOI":"10.1182\/blood-2017-10-810366","article-title":"Molecular Genetic Investigation, Clinical Features, and Response to Treatment in 21 Patients with Schnitzler Syndrome","volume":"131","author":"Rowczenio","year":"2018","journal-title":"Blood"},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Mah\u00e9vas, T., Arnulf, B., Bouaziz, J.-D., Bulai Livideanu, C., Osio, A., Servy, A., Cribier, B., Sassolas, B., Jachiet, M., and Michel, L. (2020). Plasma Cell-Directed Therapies in Monoclonal Gammopathy-Associated Scleromyxedema. Blood.","DOI":"10.1182\/blood.2019002300"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"e207","DOI":"10.3324\/haematol.2017.177444","article-title":"IGHV Segment Utilization in Immunoglobulin Gene Rearrangement Differentiates Patients with Anti-Myelin-Associated Glycoprotein Neuropathy from Others Immunoglobulin M-Gammopathies","volume":"103","author":"Allain","year":"2018","journal-title":"Haematologica"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"e827","DOI":"10.1016\/S2352-3026(20)30224-6","article-title":"Consensus Treatment Recommendations from the Tenth International Workshop for Waldenstr\u00f6m Macroglobulinaemia","volume":"7","author":"Castillo","year":"2020","journal-title":"Lancet Haematol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1182\/blood-2016-09-719773","article-title":"How I Treat Cryoglobulinemia","volume":"129","author":"Muchtar","year":"2017","journal-title":"Blood"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1159\/000445841","article-title":"Improved (4 Plus 2) Rituximab Protocol for Severe Cases of Mixed Cryoglobulinemia: A 6-Year Observational Study","volume":"43","author":"Roccatello","year":"2016","journal-title":"Am. J. Nephrol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"3128","DOI":"10.1182\/blood-2013-10-535088","article-title":"Novel Anti-B-Cell Maturation Antigen Antibody-Drug Conjugate (GSK2857916) Selectively Induces Killing of Multiple Myeloma","volume":"123","author":"Tai","year":"2014","journal-title":"Blood"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1111\/all.12129","article-title":"Schnitzler\u2019s Syndrome: Diagnosis, Treatment, and Follow-Up","volume":"68","author":"Simon","year":"2013","journal-title":"Allergy"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1111\/all.13035","article-title":"Schnitzler Syndrome: Validation and Applicability of Diagnostic Criteria in Real-Life Patients","volume":"72","author":"Gusdorf","year":"2017","journal-title":"Allergy"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.1016\/j.autrev.2014.08.031","article-title":"Long-Term Effectiveness and Safety of Interleukin-1 Receptor Antagonist (Anakinra) in Schnitzler\u2019s Syndrome: A French Multicenter Study","volume":"13","author":"Henry","year":"2014","journal-title":"Autoimmun. Rev."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1186\/2045-7022-4-41","article-title":"Schnitzler\u2019s Syndrome: Lessons from 281 Cases","volume":"4","year":"2014","journal-title":"Clin. Transl. Allergy"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"191","DOI":"10.2165\/11595240-000000000-00000","article-title":"Etiology and Management of Pyoderma Gangrenosum: A Comprehensive Review","volume":"13","author":"Ahronowitz","year":"2012","journal-title":"Am. J. Clin. Dermatol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1001\/jamadermatol.2017.5978","article-title":"The Association of Age With Clinical Presentation and Comorbidities of Pyoderma Gangrenosum","volume":"154","author":"Ashchyan","year":"2018","journal-title":"JAMA Dermatol."},{"key":"ref_39","doi-asserted-by":"crossref","unstructured":"Machan, A., Azendour, H., Frikh, R., Hjira, N., and Boui, M. (2020). The Dilemma of Treating Pyoderma Gangrenosum Associated with Monoclonal Gammopathy of Undetermined Significance. Dermatol. Online J., 26.","DOI":"10.5070\/D3265048791"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.jaad.2013.01.007","article-title":"Scleromyxedema: A Multicenter Study of Characteristics, Comorbidities, Course, and Therapy in 30 Patients","volume":"69","author":"Rongioletti","year":"2013","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1111\/cge.13865","article-title":"Cutis Laxa: A Comprehensive Overview of Clinical Characteristics and Pathophysiology","volume":"99","author":"Beyens","year":"2021","journal-title":"Clin. Genet."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1038\/sj.jid.5700047","article-title":"Inflammatory Destruction of Elastic Fibers in Acquired Cutis Laxa Is Associated with Missense Alleles in the Elastin and Fibulin-5 Genes","volume":"126","author":"Hu","year":"2006","journal-title":"J. Invest. Dermatol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1111\/j.1600-0609.2008.01181.x","article-title":"Generalized Cutis Laxa and Fibrillar Glomerulopathy Resulting from IgG Deposition in IgG-Lambda Monoclonal Gammopathy: Pulmonary Hemorrhage during Stem Cell Mobilization and Complete Hematological Response with Bortezomib and Dexamethasone Therapy","volume":"82","author":"Rovira","year":"2009","journal-title":"Eur. J. Haematol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1016\/j.jaad.2018.03.039","article-title":"Cutis Laxa Associated with Monoclonal Gammopathy: 14 New Cases and Review of the Literature","volume":"79","author":"Jachiet","year":"2018","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1192","DOI":"10.1001\/jamadermatol.2014.725","article-title":"Acquired Cutis Laxa Associated with Heavy Chain Deposition Disease Involving Dermal Elastic Fibers","volume":"150","author":"Sherman","year":"2014","journal-title":"JAMA Dermatol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1177\/120347540300700505","article-title":"Generalized Cutis Laxa Associated with Heavy Chain Deposition Disease","volume":"7","author":"Tan","year":"2003","journal-title":"J. Cutan. Med. Surg."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1002\/ajh.21603","article-title":"Systemic AL Amyloidosis with Acquired Factor X Deficiency: A Study of Perioperative Bleeding Risk and Treatment Outcomes in 60 Patients","volume":"85","author":"Thompson","year":"2010","journal-title":"Am. J. Hematol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"S2","DOI":"10.1016\/S0049-3848(13)70003-3","article-title":"Diagnosis and Treatment of Acquired von Willebrand Syndrome","volume":"130","author":"Tiede","year":"2012","journal-title":"Thromb. Res."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"2032","DOI":"10.3324\/haematol.2020.255117","article-title":"Acquired von Willebrand Syndrome: Focused for Hematologists","volume":"105","author":"Franchini","year":"2020","journal-title":"Haematologica"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1111\/bjh.15200","article-title":"Low Levels of von Willebrand Markers Associate with High Serum IgM Levels and Improve with Response to Therapy, in Patients with Waldenstr\u00f6m Macroglobulinaemia","volume":"184","author":"Castillo","year":"2019","journal-title":"Br. J. Haematol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1002\/ajh.20829","article-title":"Association of Acquired von Willebrand Syndrome with AL Amyloidosis","volume":"82","author":"Kos","year":"2007","journal-title":"Am. J. Hematol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1002\/jcla.21658","article-title":"The Effect of Paraprotein on Platelet Aggregation: Paraprotein and Platelet Aggregation","volume":"28","author":"Djunic","year":"2014","journal-title":"J. Clin. Lab. Anal."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1172\/JCI112270","article-title":"A Myeloma Paraprotein with Specificity for Platelet Glycoprotein IIIa in a Patient with a Fatal Bleeding Disorder","volume":"77","author":"DiMinno","year":"1986","journal-title":"J. Clin. Investig."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.survophthal.2004.10.002","article-title":"Immunotactoid Keratopathy: A Clinicopathologic Case Report and a Review of Reports of Corneal Involvement in Systemic Paraproteinemias","volume":"50","author":"Garibaldi","year":"2005","journal-title":"Surv. Ophthalmol."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.bbmt.2017.08.031","article-title":"High-Dose Melphalan and Stem Cell Transplantation in Patients on Dialysis Due to Immunoglobulin Light-Chain Amyloidosis and Monoclonal Immunoglobulin Deposition Disease","volume":"24","author":"Batalini","year":"2018","journal-title":"Biol. Blood Marrow Transplant."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"2679","DOI":"10.3324\/haematol.2019.239632","article-title":"Peripheral Neuropathy and Monoclonal Gammopathy of Undetermined Significance: A Population-Based Study Including 15,351 Cases and 58,619 Matched Controls","volume":"105","author":"Turesson","year":"2020","journal-title":"Haematologica"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1002\/(SICI)1097-4598(200001)23:1<73::AID-MUS9>3.0.CO;2-5","article-title":"Diagnostic Criteria for Demyelinating Polyneuropathy Associated with Monoclonal Gammopathy","volume":"23","author":"Notermans","year":"2000","journal-title":"Muscle Nerve"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1002\/ana.21577","article-title":"Placebo-Controlled Trial of Rituximab in IgM Anti-Myelin-Associated Glycoprotein Antibody Demyelinating Neuropathy","volume":"65","author":"Dalakas","year":"2009","journal-title":"Ann. Neurol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"2217","DOI":"10.1212\/WNL.0b013e318296e92b","article-title":"Placebo-Controlled Trial of Rituximab in IgM Anti-Myelin-Associated Glycoprotein Neuropathy","volume":"80","author":"Viala","year":"2013","journal-title":"Neurology"},{"key":"ref_60","first-page":"CD002827","article-title":"Immunotherapy for IgM Anti-Myelin-Associated Glycoprotein Paraprotein-Associated Peripheral Neuropathies","volume":"10","author":"Lunn","year":"2016","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1007\/s11940-017-0479-9","article-title":"Advances in the Treatment of Paraproteinemic Neuropathy","volume":"19","author":"Bianco","year":"2017","journal-title":"Curr. Treat. Options Neurol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"302","DOI":"10.3324\/haematol.2019.243139","article-title":"Chemoimmunotherapy with Rituximab, Cyclophosphamide and Prednisolone in IgM Paraproteinaemic Neuropathy: Evidence of Sustained Improvement in Electrophysiological, Serological and Functional Outcomes","volume":"106","author":"Colchester","year":"2020","journal-title":"Haematologica"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1430","DOI":"10.1056\/NEJMoa1501548","article-title":"Ibrutinib in Previously Treated Waldenstr\u00f6m\u2019s Macroglobulinemia","volume":"372","author":"Treon","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"e720","DOI":"10.1212\/NXI.0000000000000720","article-title":"The Bruton Tyrosine Kinase Inhibitor Ibrutinib Improves Anti-MAG Antibody Polyneuropathy","volume":"7","author":"Castellani","year":"2020","journal-title":"Neurol. Neuroimmunol. Neuroinflam."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1212\/WNL.54.3.615","article-title":"Distal Acquired Demyelinating Symmetric Neuropathy","volume":"54","author":"Katz","year":"2000","journal-title":"Neurology"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1016\/j.mayocp.2017.02.003","article-title":"Monoclonal Gammopathy-Associated Peripheral Neuropathy: Diagnosis and Management","volume":"92","author":"Chaudhry","year":"2017","journal-title":"Mayo Clin. Proc."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"761","DOI":"10.1002\/acr.23908","article-title":"Clinical and Molecular Phenotyping in Scleromyxedema Pretreatment and Posttreatment With Intravenous Immunoglobulin","volume":"72","author":"Mecoli","year":"2020","journal-title":"Arthritis Care Res."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"1943","DOI":"10.3324\/haematol.2019.239913","article-title":"Halting the Vicious Cycle within the Multiple Myeloma Ecosystem: Blocking JAM-A on Bone Marrow Endothelial Cells Restores Angiogenic Homeostasis and Suppresses Tumor Progression","volume":"106","author":"Solimando","year":"2020","journal-title":"Haematologica"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1056\/NEJMoa1508808","article-title":"Clonal Immunoglobulin against Lysolipids in the Origin of Myeloma","volume":"374","author":"Nair","year":"2016","journal-title":"N. Engl. J. Med."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"e041296","DOI":"10.1136\/bmjopen-2020-041296","article-title":"Health Impact of Monoclonal Gammopathy of Undetermined Significance (MGUS) and Monoclonal B-Cell Lymphocytosis (MBL): Findings from a UK Population-Based Cohort","volume":"11","author":"Lamb","year":"2021","journal-title":"BMJ Open"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"1714","DOI":"10.3324\/haematol.2009.010066","article-title":"Patterns of Survival and Causes of Death Following a Diagnosis of Monoclonal Gammopathy of Undetermined Significance: A Population-Based Study","volume":"94","author":"Kristinsson","year":"2009","journal-title":"Haematologica"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"1581","DOI":"10.3324\/haematol.2013.084830","article-title":"Schnitzler Syndrome: An under-Diagnosed Clinical Entity","volume":"98","author":"Jain","year":"2013","journal-title":"Haematologica"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1038\/s41408-021-00480-w","article-title":"Iceland Screens, Treats, or Prevents Multiple Myeloma (IStopMM): A Population-Based Screening Study for Monoclonal Gammopathy of Undetermined Significance and Randomized Controlled Trial of Follow-up Strategies","volume":"11","author":"Love","year":"2021","journal-title":"Blood Cancer J."}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/13\/20\/5131\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T07:12:40Z","timestamp":1760166760000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/13\/20\/5131"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,10,13]]},"references-count":73,"journal-issue":{"issue":"20","published-online":{"date-parts":[[2021,10]]}},"alternative-id":["cancers13205131"],"URL":"https:\/\/doi.org\/10.3390\/cancers13205131","relation":{},"ISSN":["2072-6694"],"issn-type":[{"value":"2072-6694","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,10,13]]}}}