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$Unique_ID{BRK03513}
$Pretitle{}
$Title{Banti's Syndrome}
$Subject{Banti's Syndrome Banti's Disease Hypersplenism Cirrhosis Gaucher
Disease Felty Syndrome}
$Volume{}
$Log{}
Copyright (C) 1989 National Organization for Rare Disorders, Inc.
722:
Banti's Syndrome
** IMPORTANT **
It is possible that the main title of the article (Banti's Syndrome) is
not the name you expected. Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
Banti's Disease
Hypersplenism
Information on the following diseases can be found in the Related
Disorders section of this report:
Cirrhosis
Gaucher Disease
Felty Syndrome
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Banti's Syndrome is a disorder characterized by abnormal enlargement of
the spleen resulting from a blood clot in the portal or splenic vein,
narrowing of these veins, birth abnormalities or liver disease (cirrhosis).
Symptoms
In early stages of Banti's Syndrome, symptoms 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 which in turn causes vomiting of blood and the passage of dark
stools composed of decomposing blood. Ultimately the liver itself may become
enlarged in some cases. The disorder follows a similar course to cirrhosis
of the liver. However the spleen enlargement in Banti's Syndrome is the
primary symptom.
Causes
Banti's Syndrome is caused by chronically increased pressure in the vein that
carries blood away from the spleen. This increased pressure may result from
cirrhosis of the liver, the presence of a blood clot in the portal or splenic
vein, narrowing of these veins or birth abnormalities. The spleen then
becomes enlarged due to the accumulation of blood that is unable to escape.
Related Disorders
Symptoms of the following disorders can be similar to those of Banti's
Syndrome. Comparisons may be useful for a differential diagnosis:
Cirrhosis is a chronic progressive liver disorder. Symptoms can include
an enlarged liver, fatigue, unexplained weight loss and a yellow
discoloration of the skin (jaundice). (For more information on this
disorder, choose "Primary Biliary Cirrhosis" as your search term in the Rare
Disease Database).
Gaucher Disease is an inherited metabolic disease caused by a failure to
produce the enzyme glucocerebrosidase. It is the most common of the lipid
storage disorders. Gaucher disease patients may have an enlarged spleen
and/or liver, a low blood count and bone deterioration. (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 which affects the
skin and spleen as well as the joints. Symptoms include a yellowish-brown
discoloration of the skin, enlargement of the spleen, and joint stiffness.
(For more information on this disorder, choose "Felty Syndrome" as your
search term in the Rare Disease Database).
Therapies: Standard
Treatment of Banti's disease is dependent upon the cause. Blood flow through
a narrowed vein or a vein blocked by a blood clot may be rerouted by a
surgical shunt to bypass the obstruction. The treatment of cirrhosis depends
upon the type and cause of the cirrhosis. An orphan drug for bleeding
esophageal varices was approved by the FDA in 1988. The drug, Ethamolin, is
manufactured by Glaxo pharmaceuticals.
Therapies: Investigational
This disease entry is based upon medical information available through
December 1989. Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate. Please check with the agencies listed in the Resources section for
the most current information about this disorder.
Resources
For more information on Banti's Syndrome, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
National Digestive Diseases Information Clearinghouse
Box NDDIC
Bethesda, MD 20892
(301) 468-6344
References
EVALUATION OF SPLENIC EMBOLIZATION IN PATIENTS WITH PORTAL HYPERTENSION AND
HYPERSPLENISM. A. Alwmark et al.; ANN SURG (1982 Nov) 196(5). Pp. 518-245.
THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D., ed.-in-chief;
Merck, Sharp & Dohme Laboratories., 1982. Pp. 769.