EBV-associated T-cell lymphoproliferative diseases
(EBV T-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 EBV T-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 CD2 TIA1 CD56﹣, 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 EBV T-cell LPDs
and add to the understanding of this rare disease.
Cite this paper
Li, M. (2017). EBV-Associated T-Cell Lymphoproliferative Diseases in Young Adults with Unusual Histopathological and Immunophenotypic Features. Open Access Library Journal, 4, e3951. doi: http://dx.doi.org/10.4236/oalib.1103951.
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