Banti’s syndrome

December 31, 2014

Synonyms

Overview

Banti's syndrome is a rare condition where chronic congestive spleen enlargement causes it to destroy red blood cells too early. This syndrome is characterized by abnormal enlargement of the spleen (splenomegaly) due to obstruction of blood flow in some veins and abnormally increased blood pressure (hypertension) within the veins of the liver (e.g., hepatic or portal veins), or the spleen (splenic veins).

Symptoms

Blood in vomit
Melena
Weakness
Flatulence
Diarrhea
Abdominal pain
Abdominal distress
Vague indigestion
Abdominal pain
Abdominal distress
Nosebleed
Pallor
Mild jaundice
Brown skin pigmentation
Sallow skin
Enlarged liver
Enlarged spleen
Anemia
Flapping hand tremor
Muscle wasting
Ascites
Swelling
Leukopenia
Cirrhosis of the liver
Increased portal vein hypertension

Causes

Banti's syndrome may occur due to a number of different factors causing obstruction of, and abnormally increased blood pressure (hypertension) within, certain veins of the spleen (splenic veins) or the liver (e.g., hepatic or portal veins). These may include abnormalities present at birth (congenital) of such veins, blood clots, or various underlying disorders causing inflammation and obstruction of veins (vascular obstruction) of the liver, such as cirrhosis. Increased arsenic intake has also been implicated in some cases. Also, cases have occurred in patients taking long-term azathioprine, particularly after kidney transplantation.

Diagnosis

The diagnosis of Banti's syndrome my be confirmed by a thorough clinical evaluation and a variety of specialized tests, particularly advanced imaging techniques such as a splenic venography and magnetic resonance imaging (MRI).

Treatment

Treatment of Banti's syndrome is dependent upon the cause. If a contributing factor such as arsenic or azathioprine is identified, the exposure should be stopped.

The main clinical problem is bleeding from esophageal or gastric swollen blood vessels (varices). Active bleeding may be treated with vasoconstrictor drugs or other methods used to treat portal hypertension. Recurrent bleeding may require that blood flow be rerouted by a surgical shunt. An orphan drug for bleeding esophageal varices, Ethamolin, was approved by the U.S. Food and Drug Administration (FDA) in 1988.