J Pathol Transl Med.  2023 Mar;57(2):123-127. 10.4132/jptm.2022.10.31.

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

• Robbins DJ
• Ranheim EA
• Kallan JE

• KMID: 2540498
• DOI: http://doi.org/10.4132/jptm.2022.10.31

Abstract

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. Its distinction from other EBV-positive T-cell lymphoproliferative disorders with overlapping features can be difficult, and particular diagnostic features may not be manifest until autopsy examination. We present the case of a 10-year-old boy with significant disability due to remote traumatic brain injury following non-accidental head trauma who died unexpectedly at home. Given the history of physical abuse and the potential for homicide charges, significant medicolegal implications arose with this case. Pathologic investigation ultimately revealed conclusive diagnostic features of SETLC including extensive proliferation of EBV-positive T cells in multiple organs. A natural manner of death was confirmed, thereby excluding delayed homicide related to complications of non-accidental head trauma.

Keyword

Lymphoma; T-cell; Epstein-Barr virus infections; Autopsy; Homicide

Figure

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  Fig. 1 Gross findings at autopsy. (A) Cavitary lesion in the right temporal lobe (arrow) consistent with remote blunt head trauma. (B) Pulmonary parenchyma with diffuse nodularity imparting a “cobblestone” appearance. (C) Markedly prominent pulmonary hilar and mediastinal lymphadenopathy.

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  Fig. 2 Microscopic findings, pulmonary hilar lymph node (A–C) and lung (D–G). (A) Lymph node with marked paracortical expansion and vague residual follicles. (B) Neoplastic lymphocytes with a spectrum of size and morphologic atypia. (C) Positive in-situ hybridization for Epstein-Barr virus encoded RNA in neoplastic T cells. (D) Pulmonary architectural effacement by a neoplastic lymphoid infiltrate. (E) Prominent increase in T cells by immunohistochemistry (IHC) for CD3. (F) Neoplastic T cells were predominantly positive for CD4 by IHC. (G) The majority of neoplastic T cells were negative for CD8 by IHC.

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  Fig. 3 Additional microscopic findings. (A) Gastric mucosa diffusely infiltrated by neoplastic lymphocytes with a spectrum of size and morphologic atypia. (B) Prominent increase in T cells within the gastric mucosa by immunohistochemistry (IHC) for CD3. (C) Positive in-situ hybridization for Epstein-Barr Virus encoded RNA within the neoplastic T cells in the gastric mucosa. (D) IHC for CD163 showing hemophagocytosis by histiocytes (arrows) in the bone marrow.

Reference

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