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Currently filtering by tag: Monoclonal immunoglobulin deposition disease

Light Chain Cast Nephropathy with Congo Red Staining

This biopsy was taken from a 60-year-old African American female with a history of diabetes mellitus and hypertension, who presented with acute kidney injury and a serum creatinine of 5.8 mg/dl. Workup revealed an IgA kappa monoclonal band by serum protein electrophoresis. The biopsy shows numerous PAS-pale, focally fractured tubular casts with associated cellular reaction (Fig 1). These casts frequently stained positive for Congo red (Fig 2), and focally showed apple-green birefringence upon polarization. Otherwise, Congo red stain was diffusely negative within the glomeruli, interstitium, vessels, and cytoplasm of tubular epithelium. Immunofluorescence showed kappa light chain restriction of the casts...

Pushing Glass (January 26, 2018)

An otherwise asymptomatic 65-year-old man presents for medical evaluation after 15 years of not seeing a physician. He was noted to have an abnormal D-dimer test and was referred for further evaluation to the emergency department where his serum creatinine was found to be 5 mg/dL. Urinalysis showed 2+ proteinuria and 3+ blood. A renal biopsy was ordered. The images are characteristic findings of which of the following: 1. Amyloidosis 2. Fibrillary Glomerulopathy 3. Monoclonal immunoglobulin deposition disease 4. Dense Deposit Disease 5. Lupus nephritis 6. Anti-brush border antibody disease Choice 3 - Monoclonal immunoglobulin deposition disease (MIDD) is characterized...

Light Chain Deposition Disease

An elderly patient presents to nephrology for a rising creatinine. Baseline creatinine was 2.1 mg/dl six weeks prior but is now 3.4 mg/dl. He has poorly controlled diabetes and moderately well-controlled hypertension. Work up revealed a MGUS with positive IgG Lambda. Biopsy performed for kidney injury and to determine if there is a renal component related to his MGUS (r/o MGRS). Electron microscopy revealed only irregular electron densities along the tubular basement membranes. No powdery deposits were found. Diagnosis: Immunofluorescence Features Suggestive of Light Chain Deposition Disease, Lambda Light Chain Type Interstitial Fibrosis, Mild Arteriosclerosis, Severe   Follow Up: Bone...