J Pathol Transl Med.  2018 Jan;52(1):51-55. 10.4132/jptm.2017.01.23.

An Autopsy Case of Epstein-Barr Virus–Associated Diffuse Large B-Cell Lymphoma of the Central Nervous System in an Immunocompromised Host

• Park SY
• Kim SI
• Kim H
• Lee Y
• Park SH

• KMID: 2403258
• DOI: http://doi.org/10.4132/jptm.2017.01.23

Abstract

Lymphomas arising in the central nervous system (CNS) of immunocompromised hosts are most commonly non-Hodgkin's lymphomas and are highly associated with Epstein-Barr virus (EBV). Here we report an autopsy case of EBV-associated CNS diffuse large B-cell lymphoma (DLBCL) in a host suffering from systemic lupus erythematosus who underwent immunosuppressive therapy. After autopsy, EBV-associated CNS DLBCL as well as pulmonary mixed aspergillosis and Pneumocystis jirovecii pneumonia were added to the cause of clinical manifestations of complicated pneumonia and cerebral hemorrhage in this immunocompromised patient. In conclusion, complex disease processes were revealed by autopsy in this case, indicating that the clinicopathological correlations observed through autopsy can improve our understanding of disease progression and contribute to the management of similar patients in the future.

Keyword

Autopsy; Central nervous system; Lymphoma; Epstein-Barr virus; Immunocompromised host

MeSH Terms

Aspergillosis
Autopsy*
B-Lymphocytes*
Central Nervous System*
Cerebral Hemorrhage
Disease Progression
Herpesvirus 4, Human
Humans
Immunocompromised Host*
Lupus Erythematosus, Systemic
Lymphoma
Lymphoma, B-Cell*
Pneumocystis jirovecii
Pneumonia

Figure

• 

  Fig. 1. (A) Brain computed tomography shows a round enhanced lesion (arrow) in the left frontal area. (B) The cut surface of the brain shows a relatively well-demarcated friable lesion with hemorrhage and necrosis (arrow) in the left frontal cortex and white matter. (C) On light microscopy the tumor is composed of sheets of large atypical lymphocytes with angiocentricity. (D) CD20 immunostaining reveals that neoplastic lymphocytes are robustly positive.

• 

  Fig. 2. (A) Epstein-Barr virus (EBV)-encoded small RNA in situ hybridization shows that nearly 100% of tumor cells are positive for EBV. (B) CD20 immunostaining delineates individual infiltrating tumor cells and perivascular spread of tumor cells along blood vessels. The largest distance from the margin of the main mass is approximately 3 mm. (C) An infarct was found in the right frontal lobe, demonstrating rarefied brain with vascular proliferation, foamy macrophage infiltration and perilesional gemistocytic reactive gliosis. (D) The internal capsule exhibits thick perivascular calcifications.

• 

  Fig. 3. (A) Chest posteroanterior view shows diffuse haziness of both lungs with marked interstitial and right pleural effusion. (B) Microscopically, the lung shows diffuse alveolar damage, frothy intra-alveolar spaces, detached alveolar lining cells and many macrophages. (C) Gomori methenamine silver staining reveals tiny black oval-to-round Pneumocystis jirovecii organisms (arrow) in the intra-alveolar space. (D) Lung tissue shows a cavitary lesion with a fungus ball of aspergillosis (Periodic acid–Schiff staining).

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