An unusual cause of gross hematuria and renal dysfunction in a young male

Abstract

Monoclonal immunoglobulin deposition disease (MIDD) is an uncommon disease with a peak incidence between the 5th and 6th decades of life. It is characterized by non-fibrillar, Congo red negative deposition of monoclonal immunoglobulins in various organs, including in the kidneys. MIDD can be of three types depending on the composition of the deposits, and includes light chain deposition disease (LCDD), heavy chain deposition disease and light and heavy chain deposition disease, of which LCDD is the most common. Renal involvement is a universal finding in MIDD, and is in the form of renal insufficiency, microscopic hematuria and nephrotic range proteinuria. Gross hematuria is a rare occurrence. Renal biopsy usually shows nodular sclerosing glomerulopathy on light microscopy and diffuse linear staining of glomerular and tubular basement membrane on immunofluorescence microscopy. We report a young male who presented with rapidly progressive renal failure and gross hematuria and was diagnosed as LCDD with nodular glomerulopathy and crescents on renal biopsy.