Justin Tin, 1, Hui Hing Tin, MD2, James Park, MD3, Kevin Tin, MD, MBA4 1Newtown Gastroenterology, Flushing, NY; 2Newtown Gastroenterology, Roslyn, NY; 3Maimonides Medical Center, Manhasset, NY; 4Newtown Gastroenterology, New York, NY
Introduction:
Banti's syndrome is a rare condition where the spleen prematurely destroys blood cells, leading to splenic enlargement caused by vascular obstructions in the portal and splenic veins, resulting in portal hypertension. It often manifests with ascites, jaundice, anemia, leukopenia, and gastrointestinal bleeding, distinct from hematological disorders. Banti's syndrome is more prevalent in individuals of Indian, Pakistani, and Japanese descent. Diagnosis typically involves advanced imaging and sometimes biopsy for histopathological confirmation. Here, we present a case of a young Chinese male displaying clinical features consistent with Banti's syndrome.
Case Description/Methods:
A 28-year-old Chinese immigrant male with a history of hepatitis B presents to the clinic for evaluation of cirrhosis, Patient reports having a splenectomy due to splenomegaly since childhood. He exhibits elevated liver enzymes (AST 42, ALT 55, HBs AG reactive, HBV DNA < 10) Abdominal ultrasound shows heterogeneous coarse hepatic echogenicity, reflecting parenchymal disease. Possible dilated portal veins in the liver, subsequently an MRI was performed, showing atrophy of right hepatic lobe, tortuous main portal vein. Intrahepatic periportal system replaced by innumerable heterogeneously distributed small and large caliber portal venous collaterals, heterogenous hepatic parenchyma, punctuate too small to characterize right hepatic lobe lesion likely tiny cyst and an absent spleen. A liver biopsy was performed including features of hepatoportal sclerosis/nodular regenerative hyperplasia and stainable hepatocellular iron, grade 2 which was suggestive of various etiologies of vascular/thrombotic disorders, immune-mediated disorders, paraneoplastic disorders or drug injury with no evidence of chronic hepatitis. Afterwards, a diagnosis of Banti's syndrome was made.
Discussion:
Banti's syndrome causes noncirrhotic portal hypertension and congestive splenomegaly, often leading to ascites. Its multifactorial causes include hepatitis B, coagulation problems, and toxic metal exposure. Patients present with GI bleeding and enlarged spleen. Beta-blockers are advised for portal hypertension prevention, and splenectomy may be needed for severe anemia and recurrent bleeding. Successful treatment of variceal hemorrhage can yield excellent outcomes, with potential five-year survival rates of 100%.
Figure: Abdominal MRI from the patient
Disclosures:
Justin Tin indicated no relevant financial relationships.
Hui Hing Tin indicated no relevant financial relationships.
James Park indicated no relevant financial relationships.
Kevin Tin indicated no relevant financial relationships.
Justin Tin, 1, Hui Hing Tin, MD2, James Park, MD3, Kevin Tin, MD, MBA4. P4853 - A Rare Case of Banti Syndrome: A Review of Literature, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.
