Hepatic veno-occlusive disease may be a rare characteristic of hepatic involvement in systemic amyloidosis: case report and literature review

Abstract

Systemic amyloidosis is a rare disease and patients with hepatic amyloidosis often present with hepatomegaly. Hepatomegaly can also be a feature of hepatic veno-occlusive disease (HVOD). We report here a case of systemic amyloidosis in a patient who was suspected of having HVOD. On the basis of computed tomography findings in the abdomen, HVOD was initially suspected in a 63-year-old man with the chief complaint of upper abdominal pain, ascites, and weight loss. Multiple patchy purpura and nerve symptoms were identified and these were due to amyloidosis. An increase in proteinuria and immunoglobulin κ light-chain levels, and thickening of the ventricular wall supported the diagnosis of systemic light-chain amyloidosis involving the liver, heart, kidney, skin, and nerves. This diagnosis was confirmed by histological examination of a bone marrow core biopsy with Congo red dye. Sequential treatment of bortezomib and dexamethasone led to good results in the patient. Findings of this rare case indicate that HVOD can be diagnosed without a definite history of hematopoietic stem cell transplantation or pyrrolizidine alkaloid ingestion, but more evidence is required to make an accurate diagnosis. Importantly, we speculate that HVOD is a rare characteristic of liver involvement in systemic amyloidosis.