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Monoclonal gammopathy of renal significance (MGRS) are a group of kidney disorders that present with kidney damage due to nephrotoxic monoclonal immunoglobulins (M proteins) secreted by clonal plasma cells or B cells. By definition, people with MGRS do not meet criteria for multiple myeloma or other hematologic malignancies. [1]
These diseases include monoclonal gammopathy of undetermined significance, smoldering multiple myeloma, multiple myeloma and Waldenström's macroglobulinemia. Almost 29% of monoclonal gammopathy patients may have MIDD. [5] In MIDD, these abnormal immunoglobulins, or fragments of them, are deposited along the basement membrane of the kidney.
Monoclonal gammopathy, also known as paraproteinemia, is the presence of excessive amounts of myeloma protein or monoclonal gamma globulin in the blood. It is usually due to an underlying immunoproliferative disorder or hematologic neoplasms, especially multiple myeloma. It is sometimes considered equivalent to plasma cell dyscrasia.
Some centers may offer kidney transplantation in the setting of monoclonal gammopathy of renal significance but risk of reoccurrence is common. [9] For these reasons, the role of transplantation continues to be an area of active discussion and expansion.
If there is an underlying monoclonal gammopathy, it is treated according to relevant guidelines. [1] A 51-year-old with crystal-storing histiocytosis in the left upper lip and cheek had the tumor removed in 2010. A second surgical procedure was performed to validate there were no more cells of this disease.
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A disease of gamma globulins is called a "gammopathy" (for example, in monoclonal gammopathy of undetermined significance). Brutton's agammaglobulinemia is an X-linked recessive disorder characterized by recurrent infections in the early-post natal period attributable to failure of pre-B cells to mature.
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