Systemic EBV-Positive T-cell Lymphoma of Childhood: A Case Report and Comprehensive Literature Review

Abstract

Abstract Introduction/Objective Systemic EBV-positive T-cell lymphoma of childhood (SETLC) is an extremely rare disease in which most cases have been reported in Eastern Asian countries. It is characterized by a clonal proliferation of EBV- infected cytotoxic T cells that most commonly affect immunocompetent children and young adults. Patients present with systemic disease with rapid disease progression and multi-organ failure leading to death. Here, we report a case of SETLC in an 18 year old male, and review clinicopathologic features of the entity from 12 reported cases in the literature. Methods/Case Report A previously healthy 18 year old male presented with fever, abdominal pain, and vomiting. Clinical workup revealed elevated serum EBV viral load, hepatosplenomegaly, lymphadenopathy, pancytopenia, DIC, and multi-organ failure. Peripheral blood smear and bone marrow biopsy showed increased atypical lymphocytes with CD3+, CD8+, CD56-, TIA-1+ phenotype. In addition, prominent hemophagocytosis was noted in the bone marrow. T- cell receptor beta gene rearrangement was positive by PCR. A diagnosis of SETLC was established. Despite chemotherapy, he deteriorated rapidly and passed. Results (if a Case Study enter NA) NA Conclusion SETLC is an extremely rare disease in which most cases have been reported in Eastern Asian countries. Common clinical manifestations include fever, hepatosplenomegaly, lymphadenopathy, cytopenia, elevated LFT, coagulopathy, hyperferritinemia, hemophagocytic lymphohistiocytosis, and high serum EBV viral loads. It is a systemic disease with multiple organs involvement by EBV positive T cells. Comprehensive literature review of 12 reported cases along with our case demonstrated these neoplastic T cells were positive for CD2, CD3, CD8, TIA1 or granzyme, and EBER in situ hybridization; negative for CD56 in most cases. Hemophagocytosis was a common finding, and T cell receptor gene rearrangement confirmed clonality of the neoplastic T cells in all reported cases. Currently, there is no standard treatment. Most patient died from the disease weeks to months after disease onset.