Figure 1: H&E in Systemic EBV-positive T-cell Lymphoma
Histologic sections show an enlarged lymph node with effaced architecture and expanded paracortical area by predominantly small-medium sized lymphocytes with irregular nuclear contours, variable chromatin and occasional prominent nucleoli. The atypical lymphocytes are admixed with numerous histiocytes, few plasma cells and immunoblasts. Also, obliterated subcapsular sinuses with extension into the adjacent extranodal tissue are evident. Occasional residual regressive primary/secondary follicles and open sinuses are noted. No cells with Reed-Stenberg/Hodgkin cell morphology identified. Rare mitotic and apoptotic bodies are seen. No large area of tumor cell necrosis identified. Focal emperipolesis of lymphocytes is noted within the histiocytes.
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Figure 2: CD2, CD3, CD7, & CD5 in Systemic EBV-positive T-cell Lymphoma
The atypical lymphocytes are positive for CD2, CD3 and CD7 (A-C respectively) with fewer cells positive for CD5 (D). The majority of lymphocytes are positive for CD8 with small subset positive for CD4. Per WHO, most cases occur in the setting of acute primary EBV infection are CD8 positive.
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Figure 3: PD-1 & CD20 in Systemic EBV-positive T-cell Lymphoma
PD-1 (middle panel image) highlights a subset of T-cells. In-situ hybridization probe for Epstein-Barr encoded RNA (EBER) is positive in the majority of lymphocytes. CD20 (left panel image) highlights B-cells and rare B-immunoblasts. No large cells with double expression of CD15 and CD30 seen. CD23 highlights residual dendritic meshworks. Finally, the neoplastic lymphocytes are negative for EMA or ALK reactivity further confirming the diagnosis. The histiocytes are positive for CD163 and negative for S100 and CD1a.
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Figure 4: Flow cytometry
Immunophenotyping analysis of the peripheral blood showed the presence of predominantly mature CD45bright T lymphocytes (94%) which contained mature T and B cells and only minor NK subsets. A minority distinct large cell component cell (8%) was present in this CD45bright gate. The T cell population displayed a significantly reversed CD4:CD8 ratio of 0.08:1.
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Figure 5: Molecular Studies
A clonal T-cell receptor gene rearrangement was detected consistent with a clonal T cell population, which defines this entity. Monoclonal expansion of TCR Ɣ gene by conventional PCR analysis or southern blot hybridization can be affected by the low sensitivity due to genetic polymorphism for various segments and can yield false negative results. Multiplex PCR with multiple primers pairs can provide data in this circumstance. Most lymphocytes were positive for Epstein-Barr encoded RNA (EBER) performed on the tissue sections by In-situ hybridization. Acquired isolated trisomy 2 abnormality although rare can present in ALK-negative lymphoma with anaplastic morphology.
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