Banti's syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at December 4, 2016
StartBanti's syndrome

Banti's disease, also known as Banti's syndrome, is a disease of the spleen. It causes the spleen to destroy red blood cells much more quickly than it should, which can cause a variety of problems with the patient's circulatory system.

Contents

Definition & Facts

Banti's syndrome was described in 1894 by an Italian doctor, Guido Banti. It is characterized by an enlarged spleen (splenomegaly), an organ which is located in the upper left side of the body. A healthy spleen creates red blood cells and destroys red blood cells once they get too old.

A healthy spleen also contributes to the body's immune system by fighting against infections. A person who suffers from Banti's disease has a spleen that destroys blood cells when they are still young and useful, instead of destroying the older cells. 

Symptoms & Complaints

The disease's symptoms worsen over time. Patients who are in the earliest stages of the disease suffer from anemia, which also causes weakness and a general feeling of fatigue.

Most patients suffer from a similar loss of platelets, which causes bleeding. This bleeding can also contribute to anemia. Patients often begin to experience bleeding in their throat and gastrointestinal tract. This bleeding causes blood in stool, and it can cause some patients to vomit blood. The additional blood loss from the vomiting aggravates the patient's anemia, which in turn makes the fatigue and weakness more severe.

Children who suffer from the advanced stages of the disease will appear very bloated. The disease can also strike a patient's white blood cells, which makes them more susceptible to infections. They are more likely to suffer from infections, and those infections tend to last longer and carry more severe symptoms. Finally, patients may suffer from an enlarged liver (hepatomegaly) and have cirrhosis, in which the liver is scarred.

Causes

Many different factors contribute to Banti's disease. Any condition that obstructs veins in the liver or spleen can cause the disease, as can anything that increases blood pressure in those veins (hepatic veins or portal veins in the liver or the splenic veins in the spleen).

Most of the time, Banti's disease is congenital, but it is possible to develop Banti's syndrome later in life. Many adult diseases can lead to the obstructions that cause Banti's disease, but cirrhosis is the most common, which makes it both a cause and a potential symptom of the disease.

High cholesterol is another common medical problem that can obstruct veins, which gives it the potential to contribute to Banti's disease. Some studies also link long-term consumption of arsenic to the illness, and some cases have been caused by taking azathioprine over long periods of time, especially after kidney transplants.

The connection to arsenic is the most likely reason that this disease is rare in developed nations but relatively more common in India and other developing nations, since their water often has higher arsenic levels. These factors contribute to the disease in both men and women, who are equally susceptible to the problem. 

Diagnosis & Tests

Banti's disease is fairly rare, and it shares most of its early symptoms with other, significantly more common diseases. That means that a differential diagnosis, which exists to differentiate between diseases that have similar symptoms, is usually necessary.

The process usually begins for adult patients when they experience weakness and fatigue. Blood tests usually reveal the anemia at that point, and the doctors begin to work to rule out potential causes.

Diagnostic testing relies on imaging studies that allow doctors to examine the spleen, including splenic venography and magnetic resonance imaging (MRI). These imaging studies will help detect an enlarged spleen as well as obstructions in the hepatic, portal, and splenic veins.

Treatment & Therapy

The treatment for Banti's disease depends on the cause. If a doctor catches the problem in the early stages, and it is being caused by exposure to arsenic or azathioprine, treatment involves the patient ceasing medication or avoiding arsenic. If high cholesterol or other treatable illnesses are contributing to Banti's disease, treating those problems can also stop the syndrome from developing.

Treatment becomes significantly more difficult in congenital cases or if the disease is detected later, and focuses on treating individual symptoms as they appear. Bleeding is the most dangerous symptom for the majority of patients, and it can be treated with vasoconstrictor medications. Extreme cases can be treated with a surgical shunt that reroutes the patient's blood around the problem area.

Patients who have trouble with infections due to the disease can be treated with antibiotics, and they often need a longer course of treatment than other patients. 

Prevention & Prophylaxis

Congenital cases of Banti's disease cannot be prevented. Non-congenital cases may be prevented with a healthy diet that helps to maintain a healthy blood pressure and by avoiding medications that can provoke the disease.

Patients who develop Banti's syndrome can prevent the worst of the symptoms by taking a few precautions. Since they are prone to bleeding, they should avoid situations that risk damage to the skin or internal organs. Proper hygiene and wound dressing are also important in staving off infection.