Light and heavy chain deposition disease associated with CH1 deletion.

Autor: Cohen C; Service de néphrologie , hôpital Necker , Paris , France., El-Karoui K; Service de néphrologie , hôpital Necker , Paris , France., Alyanakian MA; Service d'immunologie , hôpital Necker , Paris , France., Noel LH; Service d'anatomo-pathologie , hôpital Necker , Paris , France., Bridoux F; Service de néphrologie , CHU Poitiers , Poitiers , France., Knebelmann B; Service de néphrologie , hôpital Necker , Paris , France.
Jazyk: angličtina
Zdroj: Clinical kidney journal [Clin Kidney J] 2015 Apr; Vol. 8 (2), pp. 237-9. Date of Electronic Publication: 2015 Jan 28.
DOI: 10.1093/ckj/sfv002
Abstrakt: 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 deletion of CH1 in the both blood and kidney heavy chain. We report here, to our knowledge, the first case of γ1 LHCDD associated with a deletion of CH1.
Databáze: MEDLINE