BENTA disease
MONDO:0014645BENTA disease (B cell Expansion with N F-N:B and T cell Anergy) is a very rare congenital immune deficiency disorder. The main symptoms include spleen enlargement (splenomegalia) and frequent ear, sinus, and lung infections early in life. Some patients can present with molluscum contagiosum or chronic Epstein-Barr virus (EBV) infection. Blood exams show alterations of several immune cells with very high numbers of polyclonal B cell lymphocytos (above 2200/N<l) and few memory B cells. Other findings are low levels of IgM in blood and poor antibody responses to specific vaccines. BENTA disease is caused by mutations in the CARD11 gene. There is no established treatment, but some patients have their spleen removed and there is one case of a hematopoietic stem cell transplantation with good results.
Also known as: B-cell expansion with NF-kB and T-cell anergy disease, B-cell expansion with NFKB and T-cell anergy, BENTA
47 clinical trials for this condition and its sub-types.
Follow this condition — get notified about new trialsBroader categories
-
New shot could help Immune-Compromised patients fight infections
Disease control OngoingThis phase 3 trial is testing a new medicine called Newnorm, a concentrated antibody solution given as a shot under the skin, in 50 people with primary immunodeficiency (a condition where the immune system doesn't work properly). The goal is to see if Newnorm can prevent serious …
Phase: PHASE3 • Sponsor: Octapharma • Aim: Disease control
Last updated Jun 27, 2026 13:03 UTC
-
New antibody therapy aims to shield kids with rare immune disease from deadly infections
Disease control OngoingThis study tests a medicine called Kedrion IVIG 10% in 30 children with primary immunodeficiency, a condition where the immune system doesn't work properly. The treatment is made from donated antibodies and is given through a vein to help prevent serious bacterial infections. Res…
Phase: PHASE3 • Sponsor: Kedrion S.p.A. • Aim: Disease control
Last updated Jun 27, 2026 13:00 UTC
-
Quick-Push immune therapy studied for safety and ease
Disease control OngoingThis study looks at whether giving Cutaquig® (a medicine that helps fight infections) by a faster manual push method is safe and easy for adults with primary or secondary immunodeficiency. About 36 participants will use the rapid push method at home. The goal is to see if this ap…
Sponsor: University of Alberta • Aim: Disease control
Last updated Jun 27, 2026 12:31 UTC
-
New antibody therapy tested for long-term safety in immune-deficient patients
Disease control OngoingThis study tests the long-term safety of TAK-881, a subcutaneous immunoglobulin treatment, in 53 adults and children with primary immunodeficiency diseases (PIDD) who completed a prior study. Participants receive regular infusions every 2-4 weeks and are monitored for side effect…
Phase: PHASE3 • Sponsor: Takeda • Aim: Disease control
Last updated Jun 27, 2026 12:05 UTC
-
Scientists collect skin cells to pave way for future immune disease cures
Knowledge-focused ENROLLING_BY_INVITATIONThis study collects small skin samples from people with primary immunodeficiency or immune regulation disorders, as well as from healthy volunteers. Researchers will use these cells to create cell lines for laboratory research. The goal is to better understand immune diseases and…
Sponsor: National Institute of Allergy and Infectious Diseases (NIAID) • Aim: Knowledge-focused
Last updated Jul 04, 2026 00:00 UTC