Chapter

Light-chain and heavy-chain deposition diseases

Pierre Ronco and Pierre Aucouturier

in Rheumatology and the Kidney

Second edition

Published on behalf of Oxford University Press

Published in print April 2012 | ISBN: 9780199579655
Published online February 2013 | e-ISBN: 9780191763472 | DOI: http://dx.doi.org/10.1093/med/9780199579655.003.0117

Series: Oxford Clinical Nephrology Series

Light-chain and heavy-chain deposition diseases

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1. The diagnosis of MIDD must be suspected in any patient with the nephrotic syndrome or rapidly progressive tubule-interstitial nephritis or with echocardiographic findings indicating diastolic dysfunction and the presence of a monoclonal Ig component in the serum and/or the urine. The same combination is seen also in AL-amyloidosis, which is more often associated with the λLC isotype, whereas LCDD is associated with the κLC isotype. 2. Renal biopsy plays an essential role in the diagnosis of MIDD and of the associated dysproteinaemia because sensitive techniques, including immunofixation, fail to identify a monoclonal Ig component in 10–20% of patients with LCDD/LHCDD and approximately 40% of patients with HCDD. A serum free light-chain assay fails to detect circulating monoclonal light chain in about 10% of LCDD cases. 3. The definitive diagnosis is made by the immunohistological analysis of tissue from an affected organ, in most cases the kidney, using a panel of Ig chain-specific antibodies, including anti-κ and anti- λLC antibodies to stain the non-Congophilic deposits. 4. When the biopsy stains for a single HC isotype and does not stain for LC isotypes, the diagnosis of HCDD should be suspected, and the monoclonality of the deposits should be confirmed with anti-γ heavy-chain subclass antibodies. 5. The diagnosis of the plasma cell dyscrasia relies on bone marrow aspiration and biopsy with cell morphological evaluation and, if necessary, immunophenotyping with anti-κ and anti-λ antisera to demonstrate monoclonality. 6. The respective indications of high-dose chemotherapy with blood stem cell autografting and conventional chemotherapies are rapidly evolving because of the efficacy of proteasome inhibitors and thalidomide derivatives in myeloma patients.

Chapter.  4040 words.  Illustrated.

Subjects: Nephrology

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