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Indian J Pathol Microbiol. 2020 Jan-Mar;63(1):78-82. doi: 10.4103/IJPM.IJPM_510_19.

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

Author information

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.

Abstract

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.

KEYWORDS:

Epstein-Barr virus infection; T-cell lymphoma; hemophagocytic lymphohistiocytosis

PMID:
32031127
DOI:
10.4103/IJPM.IJPM_510_19
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