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: