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Tanita, Kay, Hoshino, Akihiro, Imadome, Ken-Ichi, Kamiya, Takahiro, Inoue, Kento, Okano, Tsubasa, Yeh, Tzu-wen, Yanagimachi, Masakatsu, Shiraishi, Akira, Ishimura, Masataka, Schober, Tilmann, Rohlfs, Meino, Takagi, Masatoshi, Imai, Kohsuke, Takada, Hidetoshi, Ohga, Shouichi, Klein, Christoph, Morio, Tomohiro and Kanegane, Hirokazu (4. February 2019): Epstein-Barr Virus-Associated γδ T-Cell Lymphoproliferative Disorder Associated With Hypomorphic IL2RG Mutation. In: Frontiers in Pediatrics, Vol. 7, 15: pp. 1-6 [PDF, 1MB]


Chronic active Epstein-Barr virus (EBV) infection (CAEBV) is an EBV-associated lymphoproliferative disease characterized by repeated or sustainable infectious mononucleosis (IM)-like symptoms. EBV is usually detected in B cells in patients who have IM or Burkitt's lymphoma and even in patients with X-linked lymphoproliferative syndrome, which is confirmed to have vulnerability to EBV infection. In contrast, EBV infects T cells (CD4+ T, CD8+ T, and γδT) or NK cells mono- or oligoclonally in CAEBV patients. It is known that the CAEBV phenotypes differ depending on which cells are infected with EBV. CAEBV is postulated to be associated with a genetic immunological abnormality, although its cause remains undefined. Here we describe a case of EBV-related γδT-cell proliferation with underlying hypomorphic IL2RG mutation. The immunological phenotype consisted of γδT-cell proliferation in the peripheral blood. A presence of EBV-infected B cells and γδT cells mimicked γδT-cell-type CAEBV. Although the patient had normal expression of CD132 (common γ chain), the phosphorylation of STAT was partially defective, indicating impaired activation of the downstream signal of the JAK/STAT pathway. Although the patient was not diagnosed as having CAEBV, this observation shows that CAEBV might be associated with immunological abnormality.

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