A type of rare immune disorder, BENTA (which means B-cell expansion with NF-jB and T-cell anergy) disease causes an increase in immune system cells. This can cause a range of symptoms, including enlargement of the lymph nodes and a higher risk of developing lymphoma later in life.
Symptoms of BENTA disease may include:
BENTA disease results from the mutation of a specific gene. While the mutation is often passed to children from one of their parents, the disorder can also occur spontaneously.
Currently, there aren’t many treatments that can effectively address BENTA disease. In many cases, doctors monitor the condition and provide medication and other treatments as needed. In the event of sinus and lung infections, doctors may prescribe antibiotics. While research is ongoing to determine whether immunosuppressive medications are beneficial, it’s not yet clear whether this treatment is effective.
Along with medications, doctors may recommend removal of the spleen in patients with BENTA disease. The spleen regulates the level of white and red blood cells, as well as platelets, but doctors aren’t sure whether removing the organ can have a positive impact on BENTA disease.