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Light-chain and heavy-chain deposition diseases 

Light-chain and heavy-chain deposition diseases
Chapter:
Light-chain and heavy-chain deposition diseases
Author(s):

Pierre Ronco

and Pierre Aucouturier

DOI:
10.1093/med/9780199579655.003.0117
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date: 27 September 2020

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.

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