Related gene: NIK
Inheritance pattern: Autosomal recessive
NF-κB-inducing kinase (NIK) deficiency is a rare immunodeficiency resulting in impaired function of T cells, B cells, and natural killer (NK) cells. T cells are generally normal in number, whereas B cells and NK cells are decreased. Affected individuals are susceptible to a wide range of infections, including opportunistic parasites such as Cryptosporidium. Development of secondary lymphatics may be impaired. NIK deficiency may be amenable to hematopoietic stem cell transplant (HSCT), though the ideal methods remain unclear.