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Banti syndrome is a disorder of the spleen, the large, gland-like organ in the upper left side of the abdomen that produces red blood cells before birth and, in newborns, removes and destroys aged red blood cells, and plays a role in fighting infection. In the case of Banti syndrome, the spleen rapidly but prematurely destroys blood cells.
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). The disorder may be due to any number of different factors causing obstruction of portal, hepatic, or splenic veins including 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.
In early stages, symptoms of Banti syndrome include weakness, fatigue, anemia , and abnormal enlargement of the spleen. As the disorder progresses, the anemia becomes more severe. The anemia may be aggravated by a bleeding esophagus that may cause vomiting of blood and the passage of dark stools composed of decomposing blood. Ultimately, in some cases, the liver itself becomes enlarged and subdivided by fibrous tissue (cirrhosis). However, the spleen enlargement in Banti syndrome is the primary symptom.
Patients with Banti syndrome bruise easily, are more likely to contract bacterial infections and carry a fever for longer periods of time.
Symptoms may also include abnormal accumulation of fluid in the abdominal cavity (ascites), weakness, fatigue; abnormally low levels of circulating red blood cells (anemia), white blood cells (leukopenia), and/or platelets (thrombocytopenia), and/or episodes of bleeding (hemorrhage) from the gastrointestinal tract.
Banti 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.
Banti syndrome affects males and females equally. It is relatively common in parts of India and Japan, but rare in Western countries. Increased arsenic levels are present in drinking water in some countries and may contribute to regional differences in incidence.
Symptoms of the following disorders can be similar to those of Banti syndrome. Comparisons may be useful for a differential diagnosis:
Primary biliary cirrhosis is a chronic, progressive liver disorder that primarily affects females and typically becomes apparent during middle age. Obstruction of the small bile ducts is accompanied by yellow discoloration of the skin (jaundice). Excessive amounts of copper accumulate in the liver, and fibrous or granular hardening (induration) of the soft liver tissue develops. Although the exact cause of primary biliary cirrhosis is unknown, possible immunological, autoimmune, genetic, and/or environmental factors are under investigation as potential causes of the disorder. (For more information on this disorder, choose "Cirrhosis, Primary Biliary" as your search term in the Rare Disease Database.)
Gaucher disease is a rare, inherited metabolic disorder in which deficiency of the enzyme glucocerebrosidase results in the accumulation of harmful quantities of certain fats (lipids) throughout the body, especially within the bone marrow, spleen and liver. The symptoms and physical findings associated with Gaucher disease vary greatly from case to case, but may include: an abnormally enlarged liver and/or spleen (hepatosplenomegaly), low levels of circulating red blood cells (anemia), low levels of platelets (thrombocytopenia), and skeletal abnormalities. (For more information on this disorder, choose "Gaucher Disease" as your search term in the Rare Disease Database.)
Felty syndrome is a rare form of rheumatoid arthritis, a disorder characterized by painful, stiff, and swollen joints. Major symptoms and physical findings of Felty syndrome include an unusually large spleen (splenomegaly) and abnormally low levels of certain white blood cells (neutophils [neutropenia]). As a result of neutropenia, affected individuals may have an increased susceptibility to certain infections. Other symptoms associated with Felty syndrome may include fatigue, fever, weight loss, and/or discoloration of patches of skin (brown pigmentation). The exact cause of Felty syndrome is unknown. It is believed to be an autoimmune disorder. (For more information on this disorder, choose "Felty Syndrome" as your search term in the Rare Disease Database.)
The diagnosis of Banti 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). During MRI, a magnetic field and radio waves are used to create cross-sectional images of targeted parts of the body.
Treatment of Banti 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.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
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McCormick PA. Banti syndrome. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:377-78.
Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:973.
Behrman RE, Kliegman RM, Arvin AM. Eds. Nelson Textbook of Pediatrics. 15th ed. W.B. Saunder Company. Philadelphia, PA; 1996:1439.
Waqar SN, Jindani S, Baig NS, et al. Banti's syndrome: case report and review of the literature. J Pak Med Assoc. 2004;54:99-101.
Pickhardt PJ, Balfe DM. Portal vein calcification and associated biliary stricture in idiopathic portal hypertension (Banti's syndrome). Abdom Imaging. 1998;23:180-82.
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Banti's disease. Who Named It? nd. 2pp.
Report last updated: 2007/08/17 00:00:00 GMT+0