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Renal involvement in plasma cell dyscrasias, immunoglobulin-based amyloidoses, and fibrillary glomerulopathies, lymphomas, and leukaemias 

Renal involvement in plasma cell dyscrasias, immunoglobulin-based amyloidoses, and fibrillary glomerulopathies, lymphomas, and leukaemias
Chapter:
Renal involvement in plasma cell dyscrasias, immunoglobulin-based amyloidoses, and fibrillary glomerulopathies, lymphomas, and leukaemias
Author(s):

Pierre Ronco

, Frank Bridoux

, and Arnaud Jaccard

DOI:
10.1093/med/9780198746690.003.0495
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date: 07 March 2021

Plasma cell dyscrasias are characterized by uncontrolled proliferation of a single clone of B cells which is responsible for the secretion of a monoclonal immunoglobulin (Ig) or Ig subunit that can deposit in tissues. They can cause a wide range of renal diseases. Light-chain amyloidosis—renal presentation is usually with proteinuria, often progressing to nephrotic syndrome. A progressive decline in renal function may occur, leading finally to endstage renal failure. Diagnosis is made by the detection of monoclonal gammopathy and free light-chain excess in the serum (90% of cases), in combination with biopsy evidence of amyloid-forming light-chain deposits. Myeloma—renal failure is found at presentation in 20% of patients, occurs in 50% at some time, and is most commonly caused by cast nephropathy, with renal biopsy typically showing ‘fractured’ casts. Chemotherapy should be introduced promptly. Light-chain, light- and heavy-chain, and heavy-chain deposition disease—collectively known as monoclonal Ig deposition diseases, present with proteinuria and renal failure. Diagnosis is by renal biopsy. Treatment strategy is based on chemotherapy (bortezomib-based regimens) followed by autologous stem cell transplantation in selected cases. Fibrillary glomerulonephritis and immunotactoid glomerulopathy—usual presentation is with nephrotic syndrome, microscopic haematuria, and hypertension. Immunotactoid glomerulopathy usually responds to chemotherapy. Cryoglobulinaemia—type II (‘essential mixed’) may present with proteinuria, haematuria, hypertension, and gradually declining renal function, or with an acute nephritic picture. Renal biopsy typically reveals membranoproliferative glomerulonephritis with massive subendothelial deposits. Treatment involves antiviral agents and/or immunosuppression. Tumour lysis syndrome—a life-threatening metabolic emergency that occurs in patients with haemopathies with high cell turnover, mostly at the onset of chemotherapy. Treatment is based on saline diuresis (if possible), rasburicase, and haemodialysis (if required).

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