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Monoclonal Gammopathy of Renal Significance and Thrombotic Microangiopathy: A Case Report
Sofia Ventura1, Raquel Cabral1, Carolina Viveiros2
1Nephrology, Hospital do Divino Espírito Santo, EPER, Ponta Delgada, PRT.
Abstract:
Monoclonal gammopathy of renal significance (MGRS) is a group of pathologies that includes all kidney disorders related to a monoclonal protein in patients without diagnostic criteria for B-cell malignancies. There are multiple MGRS-associated kidney disorders, and more are still being discovered, which makes this diagnosis challenging. The relationship between monoclonal gammopathies and thrombotic microangiopathy (TMA) is of growing interest in literature. This article describes the case of a patient with newly diagnosed MGRS, presenting with rapidly progressing kidney failure and with histologic characteristics of TMA. The patient progressed to end-stage renal disease (ESRD) despite treatment with plasmapheresis and clone-directed therapy, as is currently advised in the literature. Although rare, the association between these two entities should not be unnoticed because of patients' renal and vital prognosis.
Insights
Monoclonal gammopathy of renal significance (MGRS) can cause rapidly progressing kidney failure, often presenting as thrombotic microangiopathy (TMA). Early diagnosis and treatment are crucial, though outcomes can be poor even with standard therapies.
Area of Science:
- Nephrology
- Hematology
- Pathology
Background:
- Monoclonal gammopathy of renal significance (MGRS) encompasses kidney disorders linked to monoclonal proteins in patients without overt B-cell malignancies.
- Diagnosing MGRS is complex due to numerous associated kidney diseases, with new associations continually being identified.
- The connection between monoclonal gammopathies and thrombotic microangiopathy (TMA) is an emerging area of clinical interest.
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