Vnitr Lek 2024, 70(3):188-194 | DOI: 10.36290/vnl.2024.037

Therapy of disorders with organ dysfunctions related to "Monoclonal Gammopathy of Clinical Significance" - MGCS

Zdeněk Adam, Luděk Pour

Interní hematologická a onkologická klinika LF MU a FN Brno

Monoclonal gammopathy of clinical significance (MGCS) is an umbrella term to describe a broad spectrum of disorders with remarkable organ dysfunctions related to the underlying non-malignant B or plasma cell clone. Although the clone itself is typically very small, it is associated with diverse clinical manifestations through different mechanisms, such as monoclonal protein deposition, the biological activity of the monoclonal immunoglobulin, or angiogenic/inflammatory cytokine hyper-secretion, or the ethiopathogensis is still unknown. Some predominantly involve a single organ, commonly peripheral nerves, kidney, skin and eye, while others are systemic diseases with syndromic presentations. Recognizing the clinical features with appropriate workups, analysis of monoclonal immunoglobulin in serum and urine and free light chain analysis in serum with particular tissue biopsies, are the key to making a timely diagnosis, especially when the kidney or skin is affected. Treatment strategy is similar to multiple myeloma or Waldenström macroglobulinemia, complete remission of gammopathy is prerequisite of significant improvement of clinical symptoms and reversal of organ dysfunctions. Therapy is based on clone-directed therapy, application of high effective antiplasmocytic or antilymphocytic therapy with monoclonal antibody (anti-CD20 or anti-CD38) with high probability of total disappearing of monoclonal immunoglobulin. Other therapeutic possibility is immunomodulation with high-dose intravenous immunoglobulin 2g/kg administered in 28days interval as maintenance therapy.

Keywords: monoclonal gammopathy of clinical significance, MGCS.

Accepted: April 26, 2024; Published: May 6, 2024  Show citation