Monoclonal Gammopathy of Clinical Significance (MGCS) and Related Disorders: A Review and the Role of Imaging

Overview

Journal Diagnostics (Basel)

Specialty Radiology

Date 2024 Sep 14

PMID 39272692

Authors

Ahmed O El Sadaney

Anika Dutta

Joselle Cook

Francis I Baffour

Affiliations

Soon will be listed here.

Abstract

The term monoclonal gammopathy of clinical significance (MGCS) refers to a group of symptomatic monoclonal gammopathies that do not meet the diagnostic criteria for malignant plasma cell disorders, such as multiple myeloma or Waldenström macroglobulinemia. These symptoms are attributable to the paraneoplastic effects of monoclonal immunoglobulins that occur through diverse mechanisms. The presence of symptoms distinguishes MGCS from monoclonal gammopathy of undetermined significance, which lacks significant symptomatic presentation. The presentations of MGCS are manifold, adding to the diagnostic challenge. Clinical suspicion is key for accurate and timely diagnosis. Radiologic imaging can provide pivotal information to guide the diagnosis. In this review, we discuss MGCS from a radiology perspective and highlight pertinent imaging features associated with the disorders.

References

Le Cann M, Bouhour F, Viala K, Simon L, Tard C, Rossi C . CANOMAD: a neurological monoclonal gammopathy of clinical significance that benefits from B-cell-targeted therapies. Blood. 2020; 136(21):2428-2436. DOI: 10.1182/blood.2020007092. View

Rudolphi-Solero T, Trivino-Ibanez E, Gonzalez-Jimenez A, Ramos-Font C, Rios-Tamayo R, Rebollo-Aguirre A . Diagnostic relevance of 2-[ F]-FDG PET/CT in patients recently diagnosed with monoclonal gammopathy of undetermined significance. Hematol Oncol. 2022; 41(3):434-441. DOI: 10.1002/hon.3085. View

Rajkumar S, Dimopoulos M, Palumbo A, Blade J, Merlini G, Mateos M . International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol. 2014; 15(12):e538-48. DOI: 10.1016/S1470-2045(14)70442-5. View

Sidana S, Rajkumar S, Dispenzieri A, Lacy M, Gertz M, Buadi F . Clinical presentation and outcomes of patients with type 1 monoclonal cryoglobulinemia. Am J Hematol. 2017; 92(7):668-673. PMC: 5579826. DOI: 10.1002/ajh.24745. View

Cappelli F, Gallini C, Di Mario C, Costanzo E, Vaggelli L, Tutino F . Accuracy of 99mTc-Hydroxymethylene diphosphonate scintigraphy for diagnosis of transthyretin cardiac amyloidosis. J Nucl Cardiol. 2017; 26(2):497-504. DOI: 10.1007/s12350-017-0922-z. View

Kusunoki S, Chiba A, Tai T, Kanazawa I . Localization of GM1 and GD1b antigens in the human peripheral nervous system. Muscle Nerve. 1993; 16(7):752-6. DOI: 10.1002/mus.880160710. View

Hilal T, DiCaudo D, Connolly S, Reeder C . Necrobiotic xanthogranuloma: a 30-year single-center experience. Ann Hematol. 2018; 97(8):1471-1479. DOI: 10.1007/s00277-018-3301-1. View

Stokes M, Valeri A, Herlitz L, Khan A, Siegel D, Markowitz G . Light Chain Proximal Tubulopathy: Clinical and Pathologic Characteristics in the Modern Treatment Era. J Am Soc Nephrol. 2015; 27(5):1555-65. PMC: 4849818. DOI: 10.1681/ASN.2015020185. View

Poterucha T, Elias P, Bokhari S, Einstein A, DeLuca A, Kinkhabwala M . Diagnosing Transthyretin Cardiac Amyloidosis by Technetium Tc 99m Pyrophosphate: A Test in Evolution. JACC Cardiovasc Imaging. 2020; 14(6):1221-1231. PMC: 8113330. DOI: 10.1016/j.jcmg.2020.08.027. View

10.

Nelson C, Zhong C, Hashemi D, Ashchyan H, Brown-Joel Z, Noe M . A Multicenter Cross-Sectional Study and Systematic Review of Necrobiotic Xanthogranuloma With Proposed Diagnostic Criteria. JAMA Dermatol. 2020; 156(3):270-279. PMC: 6990734. DOI: 10.1001/jamadermatol.2019.4221. View

11.

Shi X, Hu S, Luo X, Luo M, You H, Zhu Y . CT characteristics in 24 patients with POEMS syndrome. Acta Radiol. 2015; 57(1):51-7. DOI: 10.1177/0284185114564614. View

12.

Alaggio R, Amador C, Anagnostopoulos I, Attygalle A, de Oliveira Araujo I, Berti E . The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia. 2022; 36(7):1720-1748. PMC: 9214472. DOI: 10.1038/s41375-022-01620-2. View

13.

Glazebrook K, Guerra Bonilla F, Johnson A, Leng S, Dispenzieri A . Computed tomography assessment of bone lesions in patients with POEMS syndrome. Eur Radiol. 2014; 25(2):497-504. DOI: 10.1007/s00330-014-3428-y. View

14.

Sankar P, Janamanchi V, Vondenberg J, Calabrese C . Schnitzler syndrome. BMJ Case Rep. 2023; 16(5). PMC: 10201218. DOI: 10.1136/bcr-2022-254334. View

15.

Marsh E, Keddie S, Terris-Prestholt F, DSa S, Lunn M . Early VEGF testing in inflammatory neuropathy avoids POEMS syndrome misdiagnosis and associated costs. J Neurol Neurosurg Psychiatry. 2020; 92(2):172-176. DOI: 10.1136/jnnp-2020-324012. View

16.

Sikkink L, Ramirez-Alvarado M . Cytotoxicity of amyloidogenic immunoglobulin light chains in cell culture. Cell Death Dis. 2011; 1:e98. PMC: 3032327. DOI: 10.1038/cddis.2010.75. View

17.

Merlini G, Dispenzieri A, Sanchorawala V, Schonland S, Palladini G, Hawkins P . Systemic immunoglobulin light chain amyloidosis. Nat Rev Dis Primers. 2018; 4(1):38. DOI: 10.1038/s41572-018-0034-3. View

18.

Muchtar E, Gertz M, Kyle R, Lacy M, Dingli D, Leung N . A Modern Primer on Light Chain Amyloidosis in 592 Patients With Mass Spectrometry-Verified Typing. Mayo Clin Proc. 2019; 94(3):472-483. DOI: 10.1016/j.mayocp.2018.08.006. View

19.

Campo E, Jaffe E, Cook J, Quintanilla-Martinez L, Swerdlow S, Anderson K . The International Consensus Classification of Mature Lymphoid Neoplasms: a report from the Clinical Advisory Committee. Blood. 2022; 140(11):1229-1253. PMC: 9479027. DOI: 10.1182/blood.2022015851. View

20.

Wechalekar A, Fontana M, Quarta C, Liedtke M . AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: State-of-the-Art Review. JACC CardioOncol. 2022; 4(4):427-441. PMC: 9700258. DOI: 10.1016/j.jaccao.2022.08.009. View