Thrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review
| Autor: | Eric Newman, Edward J. Filippone, Rakesh Gulati, John L. Farber, Li Li |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Male
Pathology medicine.medical_specialty Thrombotic microangiopathy Immunology Plasma cell dyscrasia Review monoclonal gammopathy of renal significance Malignancy Monoclonal Gammopathy of Undetermined Significance immune system diseases hemic and lymphatic diseases medicine Immunology and Allergy Humans Aged 80 and over business.industry atypical hemolytic and uremic syndrome Thrombotic Microangiopathies Microangiopathic hemolytic anemia RC581-607 Eculizumab Middle Aged medicine.disease thrombotic microangiopathy plasma cell dyscrasia Monoclonal eculizumab Kidney Diseases Paraproteins C3 glomerulopathies Immunologic diseases. Allergy business alternate pathway of complement Kidney disease medicine.drug |
| Zdroj: | Frontiers in Immunology Frontiers in Immunology, Vol 12 (2021) |
| ISSN: | 1664-3224 |
| Popis: | Monoclonal gammopathies result from neoplastic clones of the B-cell lineage and may cause kidney disease by various mechanisms. When the underlying clone does not meet criteria for a malignancy requiring treatment, the paraprotein is called a monoclonal gammopathy of renal significance (MGRS). One rarely reported kidney lesion associated with benign paraproteins is thrombotic microangiopathy (TMA), provisionally considered as a combination signifying MGRS. Such cases may lack systemic features of TMA, such as a microangiopathic hemolytic anemia, and the disease may be kidney limited. There is no direct deposition of the paraprotein in the kidney, and the presumed mechanism is disordered complement regulation. We report three cases of kidney limited TMA associated with benign paraproteins that had no other detectable cause for the TMA, representing cases of MGRS. Two of the cases are receiving clone directed therapy, and none are receiving eculizumab. We discuss in detail the pathophysiological basis for this possible association. Our approach to therapy involves first ruling out other causes of TMA as well as an underlying B-cell malignancy that would necessitate direct treatment. Otherwise, clone directed therapy should be considered. If refractory to such therapy or the disease is severe and multisystemic, C5 inhibition (eculizumab or ravulizumab) may be indicated as well. |
| Databáze: | OpenAIRE |
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