EBV-Associated T-Cell Lymphoproliferative Diseases in Young Adults with Unusual Histopathological and Immunophenotypic Features - Open Access Library Journal

OALibJ > Vol.4 No.10, October 2017

EBV-Associated T-Cell Lymphoproliferative Diseases in Young Adults with Unusual Histopathological and Immunophenotypic Features ()

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DOI: 10.4236/oalib.1103951 822 Downloads 2,143 Views Citations

Author(s)

Mingyang Li

Affiliation(s)

Department of Pathology and Pathophysiology, Fourth Military Medical University, Xi’an, China.

ABSTRACT

EBV-associated T-cell lymphoproliferative diseases (EBVT-cell LPDs) in non-immunocompromised hosts are a heterogenous syndrome which is challengeable for both diagnosis and treatment. Here, we report four young patients of EBVT-cell LPDs with unusual histopathological and immunophenotypic features. They presented with intermittent high fever (4/4), multiple lymphadenopathy (4/4), hepatosplenomegaly (4/4), hematochezia (2/4) and high blood EBV antibodies titer (4/4) for seven months to one year. The enteroscopic examination revealed multiple ileocecus ulcers in three of four cases. Histologically, three cases showed similar dense infiltration of polymorphic composition including variable reactive components such as plasma cells and histiocytes as well as atypical lymphocytes and one case was characterized by proliferation of monomorphic atypical lymphocytes. The infiltrating lymphocytes were medium-sized with hyperchromatic nuclei and showed mild cytologic atypia. Abundant eosinophils infiltration and formation of eosinophilic abscess were seen in all cases. Multiple foci of necrosis with granuloma were observed in lymph nodes of all cases, but hemophagocytosis was absent. The immunohistochemical staining showed that infiltrative lymphocytes were CD2TIA1CD56^﹣, suggesting cytotoxic T-cells origin, but loss of pan-T markers CD3 (2/4), CD5 (4/4) and CD7 (2/4) were frequently observed. Negativity for both CD4 and CD8 (4/4) and silent T-cell receptor (TCR) expression (3/3) were detected. EBV positivity in numerous T-cells was identified by double staining of CD3 and EBER. Three patients died within one year and one patient is alive six months after initial presentation. These unusual pathologic findings prompt us being aware of EBVT-cell LPDs and add to the understanding of this rare disease.

KEYWORDS

EBV-Associated T-Cell Lymphoproliferative Diseases, Chronic Active EBV Infection, Eosinophilic Abscess, Granuloma

Share and Cite:

Li, M. (2017) EBV-Associated T-Cell Lymphoproliferative Diseases in Young Adults with Unusual Histopathological and Immunophenotypic Features. Open Access Library Journal, 4, 1-12. doi: 10.4236/oalib.1103951.

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