Diagnosis and Management of Monoclonal Gammopathy of Clinical Significance

Overview

Journal Blood Res

Publisher Springer Nature

Specialty Hematology

Date 2022 Apr 28

PMID 35483921

Authors

Hyungwoo Cho

Affiliations

Soon will be listed here.

Abstract

The term "monoclonal gammopathy of clinical significance" (MGCS) refers to any plasma cell or B-cell clonal disorder that does not meet the current criteria for malignant disorders but produces a monoclonal protein that directly or indirectly results in organ damage. The most commonly affected organs are the kidneys, nerves, and skin. This review summarizes the current classification of MGCS and its diagnostic and treatment approaches.

Citing Articles

Concurrent presentation of proliferative glomerulonephritis with monoclonal immunoglobulin deposits and light chain proximal tubulopathy: a case report and review of the literature.

Zhang J, Liu Y, Jin F, Li J, Wang H, Ma F Front Med (Lausanne). 2025; 12:1502798.

PMID: 39958827 PMC: 11825443. DOI: 10.3389/fmed.2025.1502798.

Trabecular Bone Score as a Complementary Tool for the Assessment of Bone Mineral Density in Patients with Asymptomatic Monoclonal Gammopathies.

Drakoulidou S, Ntanasis-Stathopoulos I, Kyritsi A, Koutoulidis V, Malandrakis P, Kanellias N J Clin Med. 2024; 13(21).

PMID: 39518598 PMC: 11545904. DOI: 10.3390/jcm13216461.

Elucidation of molecular basis of osteolytic bone lesions in advanced multiple myeloma.

Shin D, Kim M, Chun S, Kim D, Lee C, Ahn K Haematologica. 2024; 109(7):2207-2218.

PMID: 38205555 PMC: 11215386. DOI: 10.3324/haematol.2023.283784.

References

Kyle R, Therneau T, Rajkumar S, Larson D, Plevak M, Offord J . Prevalence of monoclonal gammopathy of undetermined significance. N Engl J Med. 2006; 354(13):1362-9. DOI: 10.1056/NEJMoa054494. View

Bazari H, Attar E, Dahl D, Uppot R, Colvin R . Case records of the Massachusetts General Hospital. Case 23-2010. A 49-year-old man with erythrocytosis, perinephric fluid collections, and renal failure. N Engl J Med. 2010; 363(5):463-75. DOI: 10.1056/NEJMcpc1004086. View

Delanaye P, Glassock R, Pottel H, Rule A . An Age-Calibrated Definition of Chronic Kidney Disease: Rationale and Benefits. Clin Biochem Rev. 2016; 37(1):17-26. PMC: 4810758. View

Fermand J, Bridoux F, Dispenzieri A, Jaccard A, Kyle R, Leung N . Monoclonal gammopathy of clinical significance: a novel concept with therapeutic implications. Blood. 2018; 132(14):1478-1485. DOI: 10.1182/blood-2018-04-839480. View

Sayed R, Wechalekar A, Gilbertson J, Bass P, Mahmood S, Sachchithanantham S . Natural history and outcome of light chain deposition disease. Blood. 2015; 126(26):2805-10. PMC: 4732758. DOI: 10.1182/blood-2015-07-658872. View

Nasr S, Vrana J, Dasari S, Bridoux F, Fidler M, Kaaki S . DNAJB9 Is a Specific Immunohistochemical Marker for Fibrillary Glomerulonephritis. Kidney Int Rep. 2018; 3(1):56-64. PMC: 5762944. DOI: 10.1016/j.ekir.2017.07.017. View

Krause K, Bonnekoh H, Ellrich A, Tsianakas A, Wagner N, Fischer J . Long-term efficacy of canakinumab in the treatment of Schnitzler syndrome. J Allergy Clin Immunol. 2020; 145(6):1681-1686.e5. DOI: 10.1016/j.jaci.2019.12.909. View

Doneddu P, Cocito D, Manganelli F, Fazio R, Briani C, Filosto M . Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database. J Neurol Neurosurg Psychiatry. 2018; 90(2):125-132. DOI: 10.1136/jnnp-2018-318714. View

Klomjit N, Leung N, Fervenza F, Sethi S, Zand L . Rate and Predictors of Finding Monoclonal Gammopathy of Renal Significance (MGRS) Lesions on Kidney Biopsy in Patients with Monoclonal Gammopathy. J Am Soc Nephrol. 2020; 31(10):2400-2411. PMC: 7609011. DOI: 10.1681/ASN.2020010054. View

10.

Mouhieddine T, Weeks L, Ghobrial I . Monoclonal gammopathy of undetermined significance. Blood. 2019; 133(23):2484-2494. DOI: 10.1182/blood.2019846782. View

11.

Szalat R, Arnulf B, Karlin L, Rybojad M, Asli B, Malphettes M . Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy. Blood. 2011; 118(14):3777-84. DOI: 10.1182/blood-2011-05-356907. View

12.

Dispenzieri A . Monoclonal gammopathies of clinical significance. Hematology Am Soc Hematol Educ Program. 2020; 2020(1):380-388. PMC: 7727544. DOI: 10.1182/hematology.2020000122. View

13.

Schroyens W, OConnell C, Sykes D . Complete and partial responses of the TEMPI syndrome to bortezomib. N Engl J Med. 2012; 367(8):778-80. DOI: 10.1056/NEJMc1205806. View

14.

Krause K, Weller K, Stefaniak R, Wittkowski H, Altrichter S, Siebenhaar F . Efficacy and safety of the interleukin-1 antagonist rilonacept in Schnitzler syndrome: an open-label study. Allergy. 2012; 67(7):943-50. DOI: 10.1111/j.1398-9995.2012.02843.x. View

15.

Rongioletti F, Merlo G, Carli C, Cribier B, Metze D, Calonje E . Histopathologic characteristics of scleromyxedema: A study of a series of 34 cases. J Am Acad Dermatol. 2016; 74(6):1194-200. DOI: 10.1016/j.jaad.2015.12.021. View

16.

Sykes D, Schroyens W . Complete Responses in the TEMPI Syndrome after Treatment with Daratumumab. N Engl J Med. 2018; 378(23):2240-2242. DOI: 10.1056/NEJMc1804415. View

17.

Neel A, Henry B, Barbarot S, Masseau A, Perrin F, Bernier C . Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler's syndrome: a French multicenter study. Autoimmun Rev. 2014; 13(10):1035-41. DOI: 10.1016/j.autrev.2014.08.031. View

18.

Sanders P, Booker B . Pathobiology of cast nephropathy from human Bence Jones proteins. J Clin Invest. 1992; 89(2):630-9. PMC: 442896. DOI: 10.1172/JCI115629. View

19.

Gupta V, El Ters M, Kashani K, Leung N, Nasr S . Crystalglobulin-induced nephropathy. J Am Soc Nephrol. 2014; 26(3):525-9. PMC: 4341488. DOI: 10.1681/ASN.2014050509. View

20.

Cohen C, Royer B, Javaugue V, Szalat R, Karoui K, Caulier A . Bortezomib produces high hematological response rates with prolonged renal survival in monoclonal immunoglobulin deposition disease. Kidney Int. 2015; 88(5):1135-43. DOI: 10.1038/ki.2015.201. View