Randall-Type Monoclonal Immunoglobulin Deposition Disease: New Insights into the Pathogenesis, Diagnosis and Management
| Autor: | Christophe Sirac, Vincent Javaugue, Frank Bridoux, Camille Cohen, Guy Touchard, Jean-Michel Goujon, Jean-Paul Fermand, Estelle Desport, Audrey Sibille, Florent Joly |
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| Rok vydání: | 2021 |
| Předmět: |
Clinical Biochemistry
Plasma cell dyscrasia glomerular disease Disease Review Immunoglobulin light chain Pathogenesis 03 medical and health sciences 0302 clinical medicine MGRS Medicine lcsh:R5-920 business.industry monoclonal gammopathy medicine.disease Pathophysiology plasma cell dyscrasia Monoclonal gammopathy 030220 oncology & carcinogenesis Immunology medicine.symptom lcsh:Medicine (General) business 030215 immunology Rare disease Monoclonal Immunoglobulin Deposition Disease |
| Zdroj: | Diagnostics Diagnostics, Vol 11, Iss 420, p 420 (2021) |
| ISSN: | 2075-4418 |
| Popis: | Randall-type monoclonal immunoglobulin deposition disease (MIDD) is a rare disease that belongs to the spectrum of monoclonal gammopathy of renal significance (MGRS). Renal involvement is prominent in MIDD, but extra-renal manifestations can be present and may affect global prognosis. Recent data highlighted the central role of molecular characteristics of nephrotoxic monoclonal immunoglobulins in the pathophysiology of MIDD, and the importance of serum free light chain monitoring in the diagnosis and follow-up disease. Clone-targeted therapy is required to improve the overall and renal survival, and the achievement of a rapid and deep hematological response is the goal of therapy. This review will focus on the recent progress in the pathogenesis and management of this rare disease. |
| Databáze: | OpenAIRE |
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