Hepatomegaly and jaundice as the presenting symptoms of systemic light-chain amyloidosis: A case report.

BACKGROUND: Light chain (AL) amyloidosis is a plasma cell dyscrasia characterized by the pathologic production and extracellular tissue deposition of fibrillar proteins derived from immunoglobulin AL fragments secreted by a clone of plasma cells, which leads to progressive dysfunction of the affected organs. The two most commonly affected organs are the heart and kidneys, and liver is rarely the dominant affected organ with only 3.9% of cases, making them prone to misdiagnosis and missed diagnosis. CASE SUMMARY: A 65-year-old woman was admitted with a 3-mo history of progressive jaundice and marked hepatomegaly. Initially, based on enhanced computed tomography scan and angiography, Budd-Chiari syndrome was considered and balloon dilatation of significant hepatic vein stenoses was performed. However, additional diagnostic procedures, including liver biopsy and bone marrow-examination, revealed immunoglobulin kapa AL amyloidosis with extensive liver involvement and hepatic vascular compression. The disease course was progressive and fatal, and the patient eventually died 5 mo after initial presentation of symptoms. CONCLUSION: AL amyloidosis with isolated liver involvement is very rare, and can be easily misdiagnosed as a vascular disease.

Non-cirrhotic portal hypertension secondary to cholangiointestinal anastomotic stricture after choledochal cyst excision: a case report.

Background: Cystectomy accompanied by biliary system reconstruction is an important treatment option for choledochal cysts, but the risk of post-operative complications is high. The most famous long-term complication is anastomotic stricture, whereas non-cirrhotic portal hypertension secondary to cholangiointestinal anastomotic stricture is rare. Case summary: Here we report the case of a 33-year-old female patient with a type I choledochal cyst who underwent choledochal cyst excision with Roux-en-Y hepaticojejunostomy. Thirteen years later, the patient presented with severe esophageal and gastric variceal bleeding, splenomegaly, and hypersplenism. Furthermore, cholangiointestinal anastomotic stricture with cholangiectasis was identified on imaging. A pathological examination of the liver suggested intrahepatic cholestasis, but the fibrosis was mild and inconsistent with severe portal hypertension. Therefore, the final diagnosis was portal hypertension secondary to a cholangiointestinal anastomotic stricture after choledochal cyst surgery. Fortunately, the patient recovered well after endoscopic treatment and dilated cholangiointestinal anastomotic stricture. Conclusion: Choledochal cyst excision with Roux-en-Y hepaticojejunostomy is the recommended standard of care for type I choledochal cysts; however, the long-term risk of cholangiointestinal anastomotic stricture requires consideration. Moreover, cholangiointestinal anastomotic stricture can lead to portal hypertension, and the degree of elevated portal pressure may be inconsistent with the degree of intrahepatic fibrosis.

Isolated gastric variceal bleeding related to non-cirrhotic portal hypertension following oxaliplatin-based chemotherapy: A case report.

BACKGROUND: Sinusoidal obstruction syndrome has been reported after oxaliplatin-based chemotherapy, but liver fibrosis and non-cirrhotic portal hypertension (NCPH) are rarely reported. CASE SUMMARY: Here, we describe the case of a 64-year-old woman who developed isolated gastric variceal bleeding 16 mo after completing eight cycles of oxaliplatin combined with capecitabine chemotherapy after colon cancer resection. Surprisingly, splenomegaly and thrombocytopenia were not accompanied by variceal bleeding, which has been reported to have predictive value for gastric variceal formation. However, a liver biopsy showed fibrosis in the portal area, suggesting NCPH. The patient underwent endoscopic treatment and experienced no further symptoms. CONCLUSION: It is necessary to guard against long-term complications after oxaliplatin-based chemotherapy. Sometimes splenic size and platelet level may not always accurately predict the occurrence of portal hypertension.

Primary hepatic angiosarcoma manifesting as hepatic sinusoidal obstruction syndrome: A case report.

BACKGROUND: Primary hepatic angiosarcoma (PHA) is a rare malignancy with a poor prognosis. It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers, and it rapidly progresses and has a high mortality. To our knowledge, PHA has not been reported to mimic hepatic sinusoidal obstruction syndrome. Herein, we present a case of PHA manifesting as hepatic sinusoidal obstruction syndrome, diagnosed using transjugular liver biopsy, that resulted in the death of the patient. CASE SUMMARY: A 71-year-old man was admitted with the primary complaint of abdominal distension, decreased appetite, fatigue in the previous month, and loss of 10 kg of weight in the past 2 years. Both the liver and spleen were enlarged, and the liver had a medium-hard texture on percussion. Laboratory examinations were performed, and abdominal plain computed tomography (CT) and contrast-enhanced CT showed hepatomegaly and splenomegaly, as well as diffuse low-density shadows distributed in the liver and spleen. Contrast-enhanced CT revealed diffuse, hypodense, nodular or flake shadows in the liver and heterogeneous enhancement in the spleen. A transjugular liver biopsy was performed. Based on the pathology results, the patient was diagnosed with hepatic sinusoidal obstruction syndrome secondary to PHA. The patient's status further deteriorated and he developed serious hepatic failure. The patient was discharged, and died 3 d later. CONCLUSION: PHA is rare and has a poor prognosis; however, transjugular liver biopsy can be safely performed to aid in diagnosis.