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. |
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. |