CD3 and EBER double positive cells in bone marrow are a diagnostic aid for EBV-positive T-cell lymphoproliferative disorders of childhood

Abstract

We describe a case of Epstein–Barr virus (EBV)-positive T-cell lymphoproliferative disorders (EBV+ T-cell LPD) of childhood. The patient presented with high fever, jaundice, hepatosplenomegaly, pancytopenia, and disseminated intravascular coagulation (DIC). On admission, liver biopsy could not be conducted due to DIC. The small but definitive number of abnormal lymphocytes in bone marrow (BM) was indicative of malignant lymphoma or related diseases. The diagnosis of EBV+ T-cell LPD of childhood was tentatively made on the basis of the presence of CD3 and EBER double positive cells in BM. We started chemotherapy, which promptly resolved the symptoms and DIC. Later the diagnosis was confirmed by the T-cell receptor rearrangement and a single band for the EBV terminal repeat region by Southern blot analysis of BM cells. The detection of EBER-positive CD3 cells in BM is a diagnostic aid for EBV+ T-cell LPD of childhood.