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Currently filtering by tag: Amyloid

Hereditary Amyloid Neuropathy

TTR gene
Elderly female with amyloid neuropathy of the sural nerve. Cross-sections of the left sural nerve prepared from formalin-fixed paraffin-embedded (FFPE) tissue stained with Congo red show dense salmon-orange material (i.e. congophilic) predominantly around endoneurial blood vessels (Figure 1), as well as a few small accumulations within nerve fascicles, diagnostic of amyloid deposition.  The material shows apple-green birefringence with compensated polarized light microscopy (not shown), and also shows an intense bright red glow (Figure 2) against a dull background under UV light fluorescence microscopy with a Texas red excitation filter (i.e. rhodamine optics).  Co-localization to the same nerve fascicle, original magnifications: ...

Diagnose This (August 2, 2021)

Amyloid Proximal Tubulopathy
This biopsy is from a 61yo male with DM, CHF, CKD4, and an abnormal SPEP. UMOD IHC was negative. What is your diagnosis?     ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​...

ALECT2 Amyloidosis

ALECT2 Amyloidosis, arkana laboratories, renal pathology
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)

AL-type Amyloidosis, arkana laboratories, diagnose this, renal path, kidney path, nephropath, chronic kidney disease
What is your diagnosis?         ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​...

Step By Step

ANCA Disease, LECT2 Amyloid, IgG4-Related Disease, AL amyloidosis, ANCA, 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…

IgA nephropathy in renal biopsy from Arkana Laboratories
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)

Amyloidosis, ALect2-Type, arkana laboratories, pushing glass
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)

Amyloid Fibril Deposits
Answer: C Amyloid fibril deposits typically measure 8-12 nm (answer C). Immunotactoid glomerulopathy deposits 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

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...