Glomerulopathies of dysproteinemias, abnormal immunoglobulin deposition, and lymphoproliferative disorders.
The glomerulopathies associated with dysproteinemias and lymphoproliferative disorders exhibit diversity in their morphologic appearance and underlying pathophysiology. Some entities, such as amyloidosis, light-chain nephropathy, and monoclonal immunoglobulin deposition disease, are now well-recognized clinical entities. The disease processes of fibrillary glomerulonephritis and immunotactoid glomerulopathy, which may be associated with these disorders, are becoming increasingly recognized and established entities as reports on significant series of patients continue to be published. Most new information about the pathogenesis of these entities comes from structural, biochemical, and synthetic studies of individual pathogenic paraproteins. The ability to use individual pathogenic human paraproteins to recreate disease in experimental animals is a development that may allow better understanding of disease pathophysiology. The determination of effective therapeutic strategies for the management of these disorders continues to be elusive.
Alpers
C E
CE
Department of Pathology, University of Washington Medical Center, Seattle 98195.
D000818:Animals; D005921:Glomerulonephritis / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000503:physiopathology; D010265:Paraproteinemias / Q000150:complications* / Q000503:physiopathology