Hypertension, renal failure, and edema in a 38-year-old man: light chain deposition disease; a case report and review of the literature.

• DOI: 10.12860/jnp.2014.14
• Publication Date: 2014-01-01
• Authors: Sarmad Said; Chad J. Cooper; Azikiwe C. Nwosu; Jorge E Bilbao; Germán T. Hernández
• Keywords: Glomerulopathy

Monoclonal immunoglobulin deposition disease (MIDD) is a rare disease, usually manifesting between the 5(th) and 6(th) decades of life but can also occur earlier. Characteristic feature MIDD non-fibrillar, Congo red negative monoclonal immunoglobulins in various organs, including kidneys. Depending on composition deposits, classified into 3 types; light chain (LCDD), which most common form, heavy (HCDD) (LHCDD). Kidney involvement MIDD. Renal biopsy reveals nodular sclerosing glomerulopathy microscopy diffuse linear staining glomerular tubular basement membranes immunofluorescence (IF) microscopy.A 38-year-old male patient recently diagnosed with hypertension presented lower extremity edema, shortness breath, fatigue. The workup that was performed different hospital prior to this admission, demonstrated presence significant proteinuria renal failure. He intermittently dialyzed obtained, showed LCDD. Further laboratory revealed an increase IgM, kappa ß2 microglobulin chain, addition insufficiency. Bone marrow 30% plasma cells. flow cytometry test monotypic cells expressing intracytoplasmic restriction lambda ratio 35/1. diagnosis LCDD established. Treatment steroids bortezomib initiated.MIDD unusual form peak incidence around decade life, however, be found younger patients. involvement, proteinuria, hematuria, are markers initial clinical presentation. Nodular about 60% affected Early early treatment improve prognostic course