Banti's syndrome

Overview

Banti’s syndrome, also known asBanti’s disease, is a chronic condition characterized by congestion and enlargement of the spleen (splenomegaly), progressive destruction of blood cells, and portal hypertension without evidence of liver cirrhosis. First described by Italian physician Guido Banti in the late 19th century, the syndrome results from obstruction or resistance in the portal venous system, leading to backflow of blood into the spleen and gastrointestinal tract. It is more prevalent in developing countries and can lead to life-threatening complications if untreated.

Causes

Banti’s syndrome is caused by increased pressure in the portal venous system, often without cirrhosis. The exact cause can vary, and in some cases, it is idiopathic (unknown). Potential contributing factors include:

• Non-cirrhotic portal hypertension

• Thrombosis or obstruction of the portal vein

• Congenital anomalies of the portal vascular system

• Chronic infections such as schistosomiasis or tuberculosis

• Autoimmune or hematologic disorders

• Exposure to toxins or certain medications that affect the vascular endothelium

Symptoms

The clinical features of Banti’s syndrome are primarily due to portal hypertension and hypersplenism:

• Splenomegaly – an enlarged spleen often detected on physical exam or imaging

• Anemia – due to excessive destruction of red blood cells

• Leukopenia – low white blood cell count

• Thrombocytopenia – low platelet count, increasing the risk of bleeding

• Gastrointestinal bleeding – from esophageal or gastric varices

• Fatigue and weakness from chronic anemia

• Ascites – accumulation of fluid in the abdomen in advanced cases

Diagnosis

Diagnosis involves ruling out cirrhosis and confirming signs of portal hypertension and splenic involvement:

• Clinical history and physical exam noting splenomegaly and signs of anemia or bleeding

• Complete blood count (CBC) showing pancytopenia (low counts of red cells, white cells, and platelets)

• Ultrasound or CT scan of the abdomen to visualize spleen size and portal vein

• Endoscopy to detect varices in the esophagus or stomach

• Liver function tests typically show no evidence of cirrhosis

• Bone marrow biopsy may be done to rule out primary hematologic disorders

Treatment

Treatment focuses on managing the complications of portal hypertension and hypersplenism:

• Beta-blockers (e.g., propranolol) to reduce portal pressure and prevent variceal bleeding

• Endoscopic band ligation for bleeding esophageal varices

• Splenectomy – surgical removal of the spleen in cases of severe hypersplenism

• Blood transfusions and iron supplementation for anemia

• Vaccination against encapsulated bacteria prior to splenectomy to reduce infection risk

• Monitoring and managing complications such as ascites or bleeding

Prognosis

The prognosis of Banti’s syndrome depends on early recognition and management of complications:

• Good prognosis if the condition is detected early and treated appropriately

• Risk of life-threatening gastrointestinal bleeding if varices rupture

• Improved outcomes with splenectomy and proper portal hypertension control

• Lifelong monitoring may be necessary to manage anemia and prevent complications

With modern imaging, endoscopic therapies, and surgical options, patients with Banti’s syndrome can achieve favorable outcomes when managed comprehensively.