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Angioimmunoblastic T-Cell Lymphoma Presenting with an Acute Serologic Epstein-Barr Virus Profile.

Beer T, Dorion P - Hematol Rep (2015)

Bottom Line: Lymph node biopsy findings typically include effacement of nodal architecture, polymorphic infiltrate, atypical T-cells (usually CD4+/CD10+/PD1+) and prominent proliferations of high endothelial venules and follicular dendritic cells.However, this classic constellation of pathologic findings is often initially obscured by a prominence of EBV+ B-immunoblasts with or without associated peripherally circulating EBV DNA.Here we document the first reported case of an acute serologic EBV profile (VCA-IgM) in a patient with AITL, and we recommend that clinicians maintain a high index of suspicion for AITL in the appropriate clinical scenario, irrespective of Epstein-Barr related findings.

View Article: PubMed Central - PubMed

Affiliation: Department of General Internal Medicine, Geisinger Medical Center, Danville , PA, USA.

ABSTRACT
Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive peripheral T-cell lymphoma typically characterized by prominent lymphadenopathy and B-symptoms at the time of presentation, polyclonal hypergammaglobulinemia, autoimmune hemolysis and frequent but highly variable involvement of Epstein-Barr virus (EBV). Lymph node biopsy findings typically include effacement of nodal architecture, polymorphic infiltrate, atypical T-cells (usually CD4+/CD10+/PD1+) and prominent proliferations of high endothelial venules and follicular dendritic cells. However, this classic constellation of pathologic findings is often initially obscured by a prominence of EBV+ B-immunoblasts with or without associated peripherally circulating EBV DNA. Here we document the first reported case of an acute serologic EBV profile (VCA-IgM) in a patient with AITL, and we recommend that clinicians maintain a high index of suspicion for AITL in the appropriate clinical scenario, irrespective of Epstein-Barr related findings.

No MeSH data available.


Related in: MedlinePlus

Lymph node biopsy revealing marked atypia and increased vascularity (A) with atypical T-cells staining positive for CD4 (B) and CD10 (C).
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fig002: Lymph node biopsy revealing marked atypia and increased vascularity (A) with atypical T-cells staining positive for CD4 (B) and CD10 (C).

Mentions: One day later, the patient returned with high fevers and shortness of breath. He was pancytopenic and now exhibited Coomb’s positivity and a nonspecific cold agglutinin. EBV viral load had increased to 6700 IU/mL. Excisional biopsy of an enlarged left axillary lymph node revealed complete effacement of the nodal architecture, a diffuse polymorphic infiltrate with a large number of atypical CD4+/CD10+/PD1+ T-cells, prominent vascular proliferation and a meshwork of follicular dendritic cells. A few scattered cells stained CD20+, but EBV staining was negative (Figure 2). Clonal rearrangements were detected in T-cell receptor genes. This constellation of findings was diagnostic for angioimmunoblastic T-cell lymphoma (AITL).


Angioimmunoblastic T-Cell Lymphoma Presenting with an Acute Serologic Epstein-Barr Virus Profile.

Beer T, Dorion P - Hematol Rep (2015)

Lymph node biopsy revealing marked atypia and increased vascularity (A) with atypical T-cells staining positive for CD4 (B) and CD10 (C).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4508553&req=5

fig002: Lymph node biopsy revealing marked atypia and increased vascularity (A) with atypical T-cells staining positive for CD4 (B) and CD10 (C).
Mentions: One day later, the patient returned with high fevers and shortness of breath. He was pancytopenic and now exhibited Coomb’s positivity and a nonspecific cold agglutinin. EBV viral load had increased to 6700 IU/mL. Excisional biopsy of an enlarged left axillary lymph node revealed complete effacement of the nodal architecture, a diffuse polymorphic infiltrate with a large number of atypical CD4+/CD10+/PD1+ T-cells, prominent vascular proliferation and a meshwork of follicular dendritic cells. A few scattered cells stained CD20+, but EBV staining was negative (Figure 2). Clonal rearrangements were detected in T-cell receptor genes. This constellation of findings was diagnostic for angioimmunoblastic T-cell lymphoma (AITL).

Bottom Line: Lymph node biopsy findings typically include effacement of nodal architecture, polymorphic infiltrate, atypical T-cells (usually CD4+/CD10+/PD1+) and prominent proliferations of high endothelial venules and follicular dendritic cells.However, this classic constellation of pathologic findings is often initially obscured by a prominence of EBV+ B-immunoblasts with or without associated peripherally circulating EBV DNA.Here we document the first reported case of an acute serologic EBV profile (VCA-IgM) in a patient with AITL, and we recommend that clinicians maintain a high index of suspicion for AITL in the appropriate clinical scenario, irrespective of Epstein-Barr related findings.

View Article: PubMed Central - PubMed

Affiliation: Department of General Internal Medicine, Geisinger Medical Center, Danville , PA, USA.

ABSTRACT
Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive peripheral T-cell lymphoma typically characterized by prominent lymphadenopathy and B-symptoms at the time of presentation, polyclonal hypergammaglobulinemia, autoimmune hemolysis and frequent but highly variable involvement of Epstein-Barr virus (EBV). Lymph node biopsy findings typically include effacement of nodal architecture, polymorphic infiltrate, atypical T-cells (usually CD4+/CD10+/PD1+) and prominent proliferations of high endothelial venules and follicular dendritic cells. However, this classic constellation of pathologic findings is often initially obscured by a prominence of EBV+ B-immunoblasts with or without associated peripherally circulating EBV DNA. Here we document the first reported case of an acute serologic EBV profile (VCA-IgM) in a patient with AITL, and we recommend that clinicians maintain a high index of suspicion for AITL in the appropriate clinical scenario, irrespective of Epstein-Barr related findings.

No MeSH data available.


Related in: MedlinePlus