Unsuspected systemic Epstein-Barr virus–positive T-cell lymphoma of childhood diagnosed at autopsy in a potential homicide case

© 2023 The Korean Society of Pathologists/The Korean Society for Cytopathology This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/ by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. pISSN 2383-7837 eISSN 2383-7845 Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoma of childhood (SETLC) is a rare, rapidly progressive, and often fatal disease of children and young adults characterized by monoclonal expansion of EBV-positive T cells in tissues or peripheral blood following infection with EBV [1]. The disease is most prevalent in eastern Asia and has also been reported in Latin America, but it is relatively rare in western countries [2]. Patients initially experience symptoms of acute viral illness including fever and malaise with subsequent development of hepatosplenomegaly, liver failure, coagulopathy, and pancytopenia. The disease is commonly associated with hemophagocytic syndrome and leads to organ failure, sepsis, and death within days to weeks. Due to its aggressive nature, prompt diagnosis is imperative though often difficult to establish, especially given overlapping features with other EBVassociated T-cell lymphoproliferative disorders [3,4]. Given the often fatal course of SETLC, particular diagnostic features may not be manifest until autopsy examination, making consideration of this entity important in the post-mortem setting [4-7]. Consideration of such rapidly progressive, fatal diseases becomes particularly important in forensic cases with unique medicolegal connotations. We present the case of a 10-year-old boy with significant disability due to remote traumatic brain injury (TBI) following non-accidental head trauma who died unexpectedly at home. Due to his debilitated state, he experienced frequent aspiration pneumonia and reportedly exhibited respiratory distress in the weeks and days prior to his death. Given the history of physical abuse and the potential for homicide charges, significant medicolegal implications arose with this case and magnified the importance of accurately ascertaining the cause and manner of death.