The reason for defining this new entity arose from the need to differentiate patients with MG but with no lesion in any other organ from patients at risk of developing kidney disease, as these latter have a high degree of morbidity and mortality due to the severity of their kidney (and sometimes systemic) lesions induced by the monoclonal protein. 1 This monoclonal protein may be light chain, heavy chain or an intact immunoglobulin. The IKMG later redefined MGRS as a clonal proliferative disorder of B cells or plasma cells that produces a monoclonal immunoglobulin with nephrotoxic effects and that does not fulfill the hematologic criteria to start the treatment of a specific malignant cancer. It describes those disorders of MGUS with renal involvement associated with a monoclonal protein. The term monoclonal gammopathy of renal significance (MGRS) was proposed by the International Kidney and Monoclonal Gammopathy Research Group (IKMG) in 2012. The monoclonal immunoglobulins are mainly of the classes IgG, IgA or IgM. A lesion is considered to be nonmalignant or premalignant if it does not damage any target organ. It is defined as a clonal proliferative disorder of B cells or plasma cells, producing a monoclonal immunoglobulin with a monoclonal peak below 30 g/L, infiltration of anomalous plasma cells into the bone marrow less than 10% and absence of clinical evidence of myeloma, lymphoma or amyloidosis. It is more frequent at older ages, affecting about 3% of the population over 50 years of age. Monoclonal gammopathy of uncertain significance (MGUS) is the most usual form. En consecuencia, esto lleva al inicio de un tratamiento hematológico específico para detener la síntesis de la proteína monoclonal y minimizar la lesión renal y sistémica.Īlthough kidney disease is a common complication of malignant hematologic cancers, most kidney diseases are associated with nonmalignant blood dyscrasias, such as monoclonal gammopathy (MG). La detección de esta proteína monoclonal y un estudio hematológico precoz son imprescindibles, así como la necesidad de una biopsia renal para establecer el diagnóstico nefropatológico asociado. El daño renal se produce tanto por mecanismos patogénicos directos, con el depósito de la proteína monoclonal en diferentes estructuras renales, como por mecanismos indirectos, actuando como un autoanticuerpo que provoca la desregulación de la vía alternativa del complemento. ![]() Este término se aplica a un concepto introducido recientemente debido a la necesidad de diferenciar esta entidad de la gammapatía monoclonal de significado incierto, teniendo en cuenta el impacto pronóstico negativo de su alta morbilidad y mortalidad a causa de la afectación tanto renal como sistémica, llegando en ocasiones a progresar a una enfermedad renal crónica avanzada. ![]() La gammapatía monoclonal de significado renal es una entidad clínico-patológica que agrupa los trastornos renales secundarios a la secreción de una inmunoglobulina monoclonal sintetizada por un clon derivado de células B y/o células plasmáticas en un paciente sin criterios de diagnóstico de mieloma múltiple. Consequently, this then leads to the start of specific hematologic treatment to detain the production of the monoclonal protein and minimize renal and systemic injury. ![]() The detection of this monoclonal protein and an early hematologic study are essential, as is the need for a kidney biopsy to establish the associated nephropathological diagnosis. ![]() The renal damage occurs via both direct pathogenic mechanisms, with the deposition of the monoclonal protein in different renal structures, as well as indirect mechanisms, acting as an autoantibody provoking dysregulation of the alternative complement pathway. This term applies to a concept recently introduced owing to the need to differentiate this entity from monoclonal gammopathy of undetermined significance, given the negative prognostic impact of its high morbidity and mortality resulting from both renal and systemic involvement, occasionally even progressing to advanced chronic kidney disease. Monoclonal gammopathy of renal significance is a clinical–pathological entity grouping renal disorders secondary to the secretion of a monoclonal immunoglobulin synthesized by a B-cell-derived clone and/or plasma cells in a patient with no diagnostic criteria for multiple myeloma.
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