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Light and heavy chain deposition disease associated with CH1 deletion.


ABSTRACT: Light and heavy chain deposition disease (LHCDD) is a rare complication of monoclonal gammopathy. In all documented cases, LHCDD is the association of deposits of a monoclonal light chain with a normal heavy chain, especially in the kidneys. We describe here a 78-year-old woman whose renal biopsy showed nodular glomerulosclerosis, initially diagnosed as diabetic nephropathy. Detailed kidney biopsy immunofluorescence study corrected the diagnosis to ?1-?-LHCDD. Advanced immunoblot analysis showed deletion of CH1 in the both blood and kidney heavy chain. We report here, to our knowledge, the first case of ?1 LHCDD associated with a deletion of CH1.

SUBMITTER: Cohen C 

PROVIDER: S-EPMC4370302 | biostudies-literature | 2015 Apr

REPOSITORIES: biostudies-literature

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Light and heavy chain deposition disease associated with CH1 deletion.

Cohen Camille C   El-Karoui Khalil K   Alyanakian Marie-Alexandra MA   Noel Laure-Hélène LH   Bridoux Franck F   Knebelmann Bertrand B  

Clinical kidney journal 20150128 2


Light and heavy chain deposition disease (LHCDD) is a rare complication of monoclonal gammopathy. In all documented cases, LHCDD is the association of deposits of a monoclonal light chain with a normal heavy chain, especially in the kidneys. We describe here a 78-year-old woman whose renal biopsy showed nodular glomerulosclerosis, initially diagnosed as diabetic nephropathy. Detailed kidney biopsy immunofluorescence study corrected the diagnosis to γ1-κ-LHCDD. Advanced immunoblot analysis showed  ...[more]

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