1. Globular amyloidosis Case 15.1 Chapter 15 1
Miscellaneous Hepatic Disorders 1
Globular amyloidosis
Case 15.1

2. Clinical Presentation15
Miscellaneous Hepatic Disorders 2
Clinical Presentation
A 55-year-old man presented with abdominal discomfort on and off for about 1 year that had become worse over the last few months.
The patient also developed jaundice.

3. Laboratory Values Course in Hospital Total Protein: Normal AST: 36015
Miscellaneous Hepatic Disorders 3
Laboratory Values
Total Protein: Normal
AST: 360
Alk Phos: 125
Total Bilirubin: 4.0
Course in Hospital
A cholangiogram was normal and an exploratory
laparotomy was performed. The common bile duct was
dilated without calculi, the gallbladder was removed, and
a wedge biopsy of the liver was taken. The lab values
returned to normal by one month after surgery.

4. 15
Miscellaneous Hepatic Disorders 4
Pathology
The parenchyma showed distinct round to oval intrasinusoidal eosinophilic bodies that were slightly more prominent in the perivenular and midzones (a, b).
The adjacent hepatocytes showed some degree of atrophy.
Figure 15.1(a)
Figure 15.1(b)

5. 15
Miscellaneous Hepatic Disorders 5
Pathology
The portal tracts also showed distinct eosinophilic bodies (c).
Figure 15.1(c)

6. 15
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Diagnosis
Globular amyloidosis

7. 15
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Comment
Globular amyloidosis is an unusual variant of typical amyloidosis.
Instead of intrasinusoidal extracellular eosinophilic amorphous linear deposits, the amyloid forms round to oval globules within the subendothelial sinusoids and the portal tracts.
Other organs including the kidneys, adrenal glands, and spleen also show the more typical amyloid deposits in these patients, with globules only developing in the liver.
The globular amyloid does stain with Congo Red, although the birefringence takes the form of Maltese crosses.

8. 15
Miscellaneous Hepatic Disorders 8
Comment
The globular amyloid does not stain with pre-albumin, amyloid-associated (AA) protein, or light chains, and there are no distinct clinical or laboratory tests distinguishing this type of amyloidosis from the more classic amyloidosis.
In this case example the amyloidosis was an incidental finding. Unfortunately the case was lost to followup after the liver tests returned to normal.