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AL Amyloidosis

A 70 year-old female presents with nephrotic range proteinuria and a creatinine of 1.2. She was in her normal level of health until about 6 months ago when she noticed foamy urine and swelling in her ankles. She has lost 30 lbs unintentionally during the last few months. Her cardiologist said that her proteinuria does not appear to be related to the heart. A kidney biopsy is performed. The glomerulus in figure 1 is distorted and has a “washed-out” or pale appearance on PAS stain. A silver stain shows material which is non-argyrophilic (figure 2). A Congo red stain shows...

Step By Step

The biopsy is from a 65 year-old female who presented with acute renal failure and elevated creatinine up to 1.9 mg/dl.  She has non-ischemic cardiomyopathy and protein electrophoresis is positive for lambda light chains.  Serologies for ANA and rheumatoid factor are within normal limits.  A biopsy was performed to rule out paraprotein associated disease and there was a specific concern for amyloidosis, due to cardiac changes. The biopsy consisted of a good sample, contained multiple cores of tissue and more than 20 glomeruli.   The glomeruli were very unremarkable by light microscopy – loops were open, with regular contours, and no...

IgA nephropathy with something extra…

The biopsy is from a 61-year-old man with a history of intermittent microscopic hematuria for many years who presents with recent 18-pound weight loss and nephrotic syndrome.  His creatinine is mildly elevated at 1.3 mg/dL.  He has 12.5 g of proteinuria and his serum albumin is 2.6 mg/dL.  The biopsy shows diffuse mild mesangial matrix expansion with no necrosis or proliferative lesions (Fig. 1).  Immunofluorescence microscopy shows extensive granular mesangial IgA deposits (3+) (Fig. 2), compatible with IgA nephropathy.  Interestingly, the Jones methenamine silver stain also shows argyrophilic spikes involving capillary loops, which are most suggestive of spicular amyloid deposits...