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Currently filtering by tag: Light chain deposition disease

Diagnose This (July 23, 2018)

What is your diagnosis?   ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​ ​...

Peritubular Giant Cells

When giant cells surround tubules… This renal biopsy was taken from a young woman with an elevated serum creatinine (4.3 mg/dL), hematuria, and proteinuria. By light microscopy, there is a prominent giant cell reaction surrounding many of the tubules (Fig 1), the differential diagnosis of which includes so-called giant cell tubulitis (a form of tubulointerstitial nephritis associated with tubular basement membrane immune complex deposits), lupus tubulointerstitial nephritis, and monoclonal immunoglobulin deposition disease. By immunofluorescence, there is intense positivity of the tubular and glomerular basement membranes for kappa light chain (3+) with no corresponding staining for lambda light chain (Fig 2-3)....

Light Chain Deposition Disease

The immunofluorescence in cases of monoclonal immunoglobulin deposition disease is characterized by linear tubular basement membrane staining. In these cases, the positive staining is restricted to the monoclonal immunoglobulin leading to disease. The photomicrographs here show strong staining for kappa compared to lambda in a case of kappa light chain deposition disease. There is also commonly staining of the interstitium, vessels, and glomeruli. When the monoclonal immunoglobulin is a heavy chain, the immunofluorescence shows strong staining for the depositing heavy chain with little to no staining for either light chain.

Pushing Glass (June 22, 2018)

The patient is an 80 year-old male who presents with fatigue and a creatinine of 6.5 mg/dl. He was in his normal state of health until he and his grandchild went to the park on a hot afternoon. Subsequently, he began to feel ill and fatigued and went to see his physician. He has a past medical history significant for long-standing hypertension which is treated with a loop diuretic. Based on this history and images, what is the best diagnosis? A. Waldenstrom macroglobulinemia B. Light Chain Deposition Disease secondary to lymphoplasmacytic lymphoma C. Light Chain Deposition Disease secondary to small...

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...

Diagnose This! (November 13, 2017)

What is your diagnosis?     ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​...

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...

Light Chain Deposition Disease and Light Chain Proximal Tubulopathy

An elderly but previously healthy patient presents with weakness and fatigue and was found to have a creatinine of 3.0 mg/dl. His baseline Cr was 1.2 mg/dl four months prior. No history of diabetes or hypertension. Biopsy performed for kidney injury, acute versus chronic. Tubular Injury, interstitial fibrosis, and intact glomeruli. 1. Tubular Injury, interstitial fibrosis, and intact glomeruli 2. Linear staining for lambda light chains along tubular basement membranes 3. Negative staining for kappa light chains along tubular basement membranes 4. Positive Lambda staining in tubular droplets 5. Negative Kappa staining in tubular droplets Electron microscopy was negative for...

Diagnose This! (July 3, 2017)

What’s your diagnosis?     ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​ ​...