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Indian Journal of Pathology and Microbiology
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CLINICO-PATHOLOGY CONFERENCE
Year : 2020  |  Volume : 63  |  Issue : 1  |  Page : 78-82

EBV-positive T/NK-associated lymphoproliferative disorders of childhood: A complete autopsy report


1 Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada, N6A 5C1; Department of Pathology and Laboratory Medicine, London Health Sciences Centre, London, Ontario, Canada, N6A 5A5, Canada
2 Department of Pathology and Laboratory Medicine, Joseph Brant Hospital, Burlington, Ontario Canada, L7S 1W7, Canada

Correspondence Address:
Jonathan Y Keow
Department of Pathology and Laboratory Medicine, London Health Sciences Centre, Western University, 339 Windermere Rd, London, Ontario N6A 5A5
Canada
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_510_19

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Introduction: Epstein-Barr Virus (EBV)-associated systemic T-cell lymphoproliferative disorder of childhood is a rare but severe manifestation of chronic EBV infection. Despite several case reports characterizing this rare hematological neoplasm, the literature describes extensive heterogeneity in the presentation of this disease. Case presentation: Here we present a complete autopsy of a 16-year-old girl who ultimately succumbed to EBV-associated systemic T-cell lymphoproliferative disorder of childhood. Her clinical presentation demonstrated a non-specific pharyngitis with positive mono spot test, evolving into fulminant multi-organ failure, disseminated intravascular coagulopathy, sepsis, and ultimately death. Conclusions: Post-mortem findings included extensive hemorrhage, and infiltration of the liver, spleen, lymph nodes and bone marrow with neoplastic T-cells. There was extensive hemophagocytic lymphohistiocytosis (HLH) within these organs, suggesting overlap between the EBV-associated systemic T-cell lymphoproliferative disorder of childhood and EBV-associated HLH. We hope these findings provide a more comprehensive overview of several possible manifestations of EBV-associated systemic T-cell lymphoproliferative disorder of childhood.


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