Case presentation:
A 53-year-old Indian gentleman with a history of hypothyroidism,
systemic hypertension, and diabetes mellitus type II complicated by
retinopathy and presumably nephropathy under conservative and follow-up
management, presented to our hospital with a chief complaint of
jaundice. The patient noticed yellowish discoloration of his eyes and
skin that gradually progressed over one week. Other complaints included
generalized, intermittent pruritus that started one month before and was
more noticeable in the palms and soles, in addition to passing
dark-colored urine.
There was no history of abdominal pain, nausea, vomiting, fever, weight
loss, or altered bowel habits. The patient is a never smoker with no
history of alcohol consumption or use of any kind of recreational drugs.
There was no history of recent travel or change in dietary habits. He
worked as a truck driver. Family history was negative. His home
medications were sodium bicarbonate, linagliptin, levothyroxine,
irbesartan, hydralazine, and furosemide.
Physical examination revealed generalized jaundice in the skin and
sclerae. No organomegaly.
Laboratory workup revealed direct hyperbilirubinemia and elevated
alkaline phosphatase and aminotransferases Table 1. The patient was
admitted as a case of painless jaundice for investigation. Ultrasound of
the abdomen and Magnetic resonance cholangiopancreatography (MRCP)
revealed cholecystolithiasis with unremarkable liver texture, no
intrahepatic and extrahepatic duct dilation.
The importance of identifying this disease is to avoid toxic
chemotherapy when it is diagnosed as AL amyloidosis. This case is to
raise awareness about this type of amyloidosis and the possibility of
severe hepatic disease presentation.
As the patient’s liver and kidney function tests were gradually
worsening over one week, this prompted more invasive investigations.
Endoscopic ultrasound was done to rule out a pancreatic mass that was
not detected on MRCP and revealed a lymph node with a size of 18x15 mm
at the porta hepatis. Fine needle aspiration biopsy of the lymph node
showed reactive lymphocytosis otherwise not suggestive of a specific
pathology.
A percutaneous ultrasound-guided liver biopsy was done. Unfortunately,
the liver biopsy was complicated by acute hemoperitoneum which
necessitated admission into the medical intensive care unit and
initiation of hemodialysis due to acute worsening of his renal function.
The liver biopsy revealed prominent globular eosinophilic deposits, seen
in the portal tracts and the sinusoids. These deposits were positive for
Congo red staining, displaying apple-green birefringence. Background
cholestasis was noted. The appearances were in keeping with globular
hepatic amyloid, which is considered to be highly sensitive and specific
for LECT2 amyloidosis (4) Figure 1, 2.
After disclosure of the diagnosis to the patient and explanation of the
nature of the disease and its progressive course, he chose to go back to
his home country as he wanted to spend more time with his family. He was
discharged from our hospital after a four-week course in a stable
condition.
We were not able to follow the patient after his travel.
Table 1: Lab investigations: