Padeira, GLAraújo, CCordeiro, AIFreixo, JMartins, CGNeves, JF2021-05-192021-05-192021Front Immunol . 2021 Apr 28;12:654167.http://hdl.handle.net/10400.17/3693In immunocompromised patients, EBV may elicit B-cell transformation and proliferation. A 5-year-old microcephalic boy was admitted with fever and non-malignant polymorphic T-cell lymphoproliferative disease associated with EBV. A presumptive diagnosis of primary immunodeficiency with inability to control EBV was made and next-generation sequencing led to the identification of a novel ZBTB24 mutation (ICF2-syndrome). This case shows that susceptibility to EBV seems to be particular of ICF-2 as it has not been described in the other types of ICF. It is mandatory to raise the hypothesis of an underlying PID in case of severe EBV infection.engEBVICF-2Primary immune deficienciesLymphoproliferationCase reportHDE PEDjournal article10.3389/fimmu.2021.654167