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Review
. 2021 Oct 13;13(20):5131.
doi: 10.3390/cancers13205131.

Treatment of Patients with Monoclonal Gammopathy of Clinical Significance

David F Moreno  1   2 , Laura Rosiñol  1   2 , María Teresa Cibeira  1   2 , Joan Bladé  1   2 , Carlos Fernández de Larrea  1   2
Affiliations
Review

Treatment of Patients with Monoclonal Gammopathy of Clinical Significance

David F Moreno et al. Cancers (Basel). .

Abstract

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.

Keywords: MGCS; MGUS; bleeding; ocular; skin.

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Conflict of interest statement

J.B.: Honoraria for lectures from Janssen, Celgene, Amgen, Takeda, and Oncopeptides. L.R.: Consulting fees from Amgen, Celgene, Sanofi, Janssen, and Takeda. C.F.d.L.: Advisory boards from Amgen, Janssen, and BMS; research grants from Janssen, BMS, Takeda, and Amgen; honoraria for lectures: BMS, Takeda, Sanofi, Amgen, Janssen, GSK, and Beigene. M.T.C.: Honoraria from Amgen and Janssen. D.F.M. declares no conflict of interest. This review was presented by Joan Bladé in the 24th European Hematology Association Congress (Amsterdam, 14 June 2019).

Figures

Figure 1
Figure 1
Patient diagnosed with multiple myeloma and type 1 cryoglobulinemia, causing severe skin ulceration on lower extremities. (A) Skin ulcers before daratumumab. (B) Improvement of the skin condition during treatment. (C) Complete resolution of the skin ulcers with daratumumab.
Figure 2
Figure 2
A 78-year-old patient with urticarial lesions, fever, mild leukocytosis, elevated CRP, and positive immunofixation for IgM kappa. The patient was diagnosed with Schnitzler syndrome.
Figure 3
Figure 3
Skin ulcer on left ankle with violaceous elevated borders in a patient with a long history of pyoderma gangrenosum and IgA-kappa monoclonal gammopathy of undetermined significance.
Figure 4
Figure 4
Rigid sclerodermoid lesions on right arm and shoulder in a patient with IgG kappa monoclonal gammopathy.
Figure 5
Figure 5
A 52-year-old patient that complaint of premature aging. Skin looks inelastic and pendulous on the neck. Immunofixation was positive for IgG-lambda. Skin biopsy was consistent with cutis laxa.
Figure 6
Figure 6
Images of corneal kappa light chain deposition disease. (A) Peripheral corneal deposits at diagnosis. (B) Three years later, the ocular examination revealed increased corneal involvement. (C) Picture taken before the autologous stem cell transplant (ASCT) showing stable disease (D) One year after ASCT, the patient achieved stringent complete response with stabilized corneal involvement.
See this image and copyright information in PMC

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