Amyloidosis

 Amyloid deposits are congophilic and show “apple green” birefringence under polarized light


image Birefringence is best demonstrated by turning light to maximum and pulling color filters out


• Immunohistochemistry
image P glycoprotein: Present in all cases, can help in diagnosis

image Light chains, SAA, and LECT2: Helps in further classification




Ancillary Tests




• Electron microscopy
image Central electron-lucent core and nonbranching fibrils of indefinite length with mean diameter of 10 nm

• Laser microdissection and mass spectrometry
image Most accurate method for identification of amyloid subtype

image Paraffin-embedded tissue or cytology material can be used


Top Differential Diagnoses




• Monoclonal immunoglobulin deposit disease

image
Vessel Wall Amyloid
Deposits are seen in the hepatic arteriole image and portal vein image in the vascular pattern of hepatic amyloidosis. This distribution is characteristic, but not specific, for the AA form.


image
Congo Red Stain
Congo red stain highlights the vascular distribution of amyloid deposits in the hepatic arteriole image and portal vein image .

image
Sinusoidal Amyloid
Congo red stain highlights the diffuse sinusoidal pattern image of hepatic amyloidosis. The amyloid deposits are compressing the hepatocytes image, leading to hepatic plate atrophy.

image
Globular Amyloid
Amyloid deposits image stain with trichrome stain, but it is often a paler staining than that seen with collagen image .


TERMINOLOGY


Definitions




• Heterogeneous disease characterized by deposition of glycoprotein fibrils in extracellular matrix and vessel walls
image Deposits composed of low molecular weight subunits (5-25 KDa) derived from normal serum proteins

• Liver involvement may be seen in different types
image Primary, or AL, amyloidosis
– Deposits are composed of fragments of monoclonal light chains

– Occurs alone or associated with other hematologic diseases (plasmacytoma, multiple myeloma Waldenstrom macroglobulinemia)

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Amyloidosis

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