Combined immunodeficiency due to OX40 deficiency

What is Combined immunodeficiency due to OX40 deficiency?

Combined immunodeficiency due to OX40 deficiency is a rare combined T and B cell immunodeficiency characterized by susceptibility to develop an aggressive, childhood-onset, disseminated, cutaneous and systemic Kaposi sarcoma.

Other condition names

  • Combined immunodeficiency with childhood-onset Kaposi sarcoma
  • Combined immunodeficiency with impaired immunity to HHV-8
  • Combined immunodeficiency with impaired immunity to human herpes virus 8

Inheritance type

Autosomal recessive

Prevalence

  • Worldwide: <1 in 1,000 000

Age of Onset

  • Childhood
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.