Renal manifestations of plasma cell dyscrasias: an appraisal from the patients' bedside to the research laboratory.
One of the most prominent features of plasma cell dyscrasias is the frequent occurrence of renal dysfunction. Renal insufficiency is a common finding with elevated serum creatinine in more than 50% of patients with multiple myeloma at the time of diagnosis. Renal failure is the second most common cause of death in myeloma surpassed only by infections. The reasons for renal failure are multifactorial and early accurate diagnosis of the renal alterations may significantly impact morbidity and survival. Renal failure may result from selective glomerular, tubular interstitial, or vascular pathology or from a combination of pathologic events. The disorders associated with plasma cell dyscrasias include those characterized by monoclonal light chain deposition, encompassing AL-amyloidosis, in addition to the less well-characterized entities, such as heavy chain deposition disease and heavy chain amyloidosis. Therefore, it is more accurate to refer to them as monoclonal immunoglobulin deposition diseases. Staining of renal biopsy specimens for kappa and lambda light chains using immunofluorescence techniques and more sophisticated advanced diagnostic techniques such as immunoelectron microscopy permit detailed characterization of the various renal pathologic manifestations. Renal biopsies can identify monoclonal immunoglobulin deposition, and nephrologists have an opportunity to detect an underlying plasma cell dyscrasia early in its clinical course before overt hematologic alterations become manifest and irreversible renal damage has occurred. The overall spectrum of clinical and pathologic manifestations of monoclonal immunoglobulin deposition renal diseases has expanded considerably in recent years. Recent developments in the research arena promise new therapeutic interventions aimed at avoiding or ameliorating renal damage and even promoting reversal of some of the pathologic alterations. Currently, the 5-year survival rate in myeloma is 29% in white patients and 30% in African-American patients, a rather modest improvement from 24% in the 1970s. Bone marrow ablation followed by transplantation is available as an alternative mode of therapy that may be extraordinarily helpful in a subset of patients with early myeloma.
Department of Pathology, Medicine and Cell Biology and Anatomy, Louisiana State University Health Sciences Center, Shreveport 71130, USA.
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Herrera
D000686:Amyloidosis / Q000209:etiology; D000911:Antibodies, Monoclonal; D016465:Bone Marrow Purging; D016026:Bone Marrow Transplantation; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D007678:Kidney Glomerulus / Q000276:immunology / Q000473:pathology; D007680:Kidney Neoplasms / Q000150:complications* / Q000473:pathology / Q000628:therapy; D007684:Kidney Tubules / Q000276:immunology / Q000473:pathology; D016253:Microscopy, Immunoelectron; D009101:Multiple Myeloma / Q000150:complications; D010265:Paraproteinemias / Q000150:complications* / Q000473:pathology / Q000628:therapy; D051437:Renal Insufficiency / Q000209:etiology*; D015996:Survival Rate
D000911:Antibodies, Monoclonal; D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains