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Review
. 2021 Jul 1;96(7):846-853.
doi: 10.1002/ajh.26155. Epub 2021 Mar 25.

The evaluation and management of monoclonal gammopathy of renal significance and monoclonal gammopathy of neurological significance

Jorge J Castillo  1 , Natalie S Callander  2 , Muhamed Baljevic  3 , Douglas W Sborov  4 , Shaji Kumar  5
Affiliations
Review

The evaluation and management of monoclonal gammopathy of renal significance and monoclonal gammopathy of neurological significance

Jorge J Castillo et al. Am J Hematol. .

Abstract

Despite the benign nature of monoclonal gammopathy of undetermined significance (MGUS), mounting data are associating MGUS with the development of organ dysfunction, specifically monoclonal gammopathy of renal significance (MGRS) and monoclonal gammopathy of neurological significance (MGNS), which could be associated with substantial morbidity. Emerging evidence suggests that patients with MGRS and MGNS could benefit from treatments used for myeloma, Waldenström 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.

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Conflict of interest statement

J.J.C. has received research funds and/or honoraria from Abbvie, Beigene, Janssen, Millennium, Pharmacyclics, Roche and T.G. Therapeutics. D.W.S. has received honoraria from G.S.K., Sanofi, SkylineDx, Legend Biotech and Janssen. S.K. has received research funds and/or honoraria from Abbvie, Allogene, Beigene, B.M.S., Celgene, Cellectar, G.S.K., MedImmune, Oncopeptides, Roche, Takeda and Tenebio. N.S.C. has no conflicts of interest to disclose.

Figures

FIGURE 1
FIGURE 1
Proposed algorithm for renal biopsy in patients with monoclonal gammopathy of renal significance. Adapted from. 4 AKI, acute kidney injury; MGRS, monoclonal gammopathy of renal significance
FIGURE 2
FIGURE 2
Proposed algorithm for evaluation of patients with monoclonal gammopathy of neurological significance. Adapted from. 34 MAG, myelin‐associated glycoprotein; MGNS, monoclonal gammopathy of neurological significance; NCS, nerve conduction studies; PN, peripheral neuropathy; POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes
See this image and copyright information in PMC

References

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