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Currently filtering by tag: Amyloid

ALECT2 Amyloidosis

The patient is a 75-year-old Hispanic male who presents with progressively worsening kidney function and a creatinine of 1.6 mg/dl (baseline 1.0 mg/dl). His physician called for a biopsy due to a slowly "creeping" creatinine over the last 8 months. Images 1 & 2 show "apple-green birefringence" on a Congo red stain under polarized light within the interstitium. Image 3 shows mild interstitial fibrosis. Image 4 shows a normal glomerulus on PAS stain and Image 5 shows positive immunohistochemical staining with Lect2. Leukocyte cell-derived chemotaxin 2 (ALECT2) amyloidosis preferentially affects Hispanics (particularly Mexicans) as well as Punjabis, First Nations people...

Diagnose This (April 1, 2019)

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

Step By Step

digging deeper, Dr. Messias, Arkana Laboratories, pathology diseases
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...

Pushing Glass (April 6, 2018)

A 75-year-old Hispanic male presents with a creatinine of 3. His creatinine has slowly been creeping up over the last year from 1.8 to the current level. No hematuria or proteinuria is reported. Serologies for ANA, dsDNA, rheumatoid factor, hepatitis B, hepatitis C, and ASO are negative. Complement levels are normal. SPEP and UPEP are within normal limits. What is the best diagnosis? A. Arterionephrosclerosis B. Amyloidosis, AL-Type C. Amyloidosis, AA-Type D. Amyloidosis, ALect2-Type   The best answer is D (Amyloidosis, ALect2-Type). From low power, the glomeruli have an ischemic appearance and the arteries have severe arteriosclerosis. However, the Congo...

Twitter Poll (September 12, 2017)

Answer: C Amyloid fibrils typically measure 8-12 nm (answer C). The deposits seen in Immunotactoid glomerulopathy are composed by tubules ranging from 20-90 nm (answer A).  The deposits from Cryoglobulin and Fibrillary glomerulopathy vary from 10-30 nm (answer B). Fibronectin glomerulopathy consists of fibril deposits measuring 14-16 nm (answer D).

AA Amyloid

This biopsy from a patient with massive proteinuria showed diffuse and near-global involvement of glomeruli by amorphous deposits (Fig. 1) which were Congo red positive (not shown). The amyloid subtype was confirmed as AA amyloid using immunohistochemistry (Fig. 2). Immunofluorescence studies showed no evidence of kappa or lambda light chain restriction within the deposits (not shown). AA amyloidosis is usually diagnosed in the setting of a prolonged inflammatory condition along with high levels of serum amyloid A (SAA) precursor protein, from which the AA amyloid fibril protein is derived. Since the early twentieth century, the incidence of AA amyloidosis has decreased...

Pushing Glass (July 25, 2017)

The patient is a 64-year-old female who presents with 4.5 grams of proteinuria, hematuria, and a creatinine of 1.5. She has a history of hypertension and coronary artery disease. What is the best diagnosis? A. Arterionephrosclerosis B. Focal Segmental Glomerulosclerosis C. Amyloidosis D. Fibrillary Glomerulonephritis The best answer is C: Amyloidosis. The biopsy, at first glance, looks like a subcapsular scar with diffuse global glomerulosclerosis. In this setting, arterionephrosclerosis would provide a good explanation for this distribution of fibrosis especially with the severe arteriosclerosis seen in the vessel. FSGS is also a consideration and can produce segmental scars with extensive...

Congo Red Stain

The ability to detect amyloid deposits in human tissue is the critical first step in making a diagnosis of amyloidosis. David P. Steensma, MD, offers readers a fascinating history of the Congo red stain (see references), which still remains the most widely used and cost-effective way to identify amyloid deposits in pathology specimens. Although reportedly first used to stain human tissue in 1886 (see H. Greisbach), it would not be until 1922 that the dye’s specificity for amyloid would be exploited (see H. Bennhold). And it would be another five years before the well-known “apple-green” birefringence of Congo red-stained amyloid deposits...