We report a non-consanguineous family from North-west India in which 3 siblings succumbed to a rare variant of combined immunodeficiency. All three had similar clinical and immunological profiles. However, the youngest child also developed Non-Hodgkin lymphoma in infancy. Genetic analysis revealed a previously reported variant in the T cell receptor alpha constant (TRAC) gene.
Keywords: Non-Hodgkin lymphoma; Rearrangements; Severe combined immunodeficiency; T cell receptor alpha constant gene.