Limits...
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

Peripheral Epstein-Barr virus data reported in cases of angioimmunoblastic T-cell lymphoma (n=44).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4508553&req=5

fig003: Peripheral Epstein-Barr virus data reported in cases of angioimmunoblastic T-cell lymphoma (n=44).

Mentions: Angioimmunoblastic T-cell lymphoma has been consistently reported to have an association with EBV. In fact, EBV-positive B-immunoblasts are detected in the lymph nodes and bone marrow in most cases of AITL.17 However, the nature of the relationship between EBV and AITL is unclear. Some authors argue that the presence of EBV reflects the profound immunodeficient state that AITL creates, while others argue that EBV itself drives the development of AITL.18,19 There have been several reports of EBV-associated B-cell proliferations in patients with AITL.20,21 In one large scale prospective series, Delfau-Larue et al. demonstrated that the presence of circulating EBV DNA is strongly correlated with the presence of circulating AITL tumor cells and that higher levels of peripheral EBV DNA at initial presentation is associated with poorer response to typical treatments.22 However, it seems that AITL has never been reported to present with an acute EBV serologic profile like that of our patient. Among the 1249 reported cases of AITL we reviewed, data on peripheral EBV DNA or serologic testing was reported in 44 cases (including 3 case series and 14 individual case reports). Among these, EBV DNA was detected in 48% (13/27), VCA-IgG was detected in 89% (31/35), EBNA-IgG was detected in 81% (26/32), but detectable EBV-IgM was not reported in a single case (0/33) (Figure 3).22-35


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

Beer T, Dorion P - Hematol Rep (2015)

Peripheral Epstein-Barr virus data reported in cases of angioimmunoblastic T-cell lymphoma (n=44).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig003: Peripheral Epstein-Barr virus data reported in cases of angioimmunoblastic T-cell lymphoma (n=44).
Mentions: Angioimmunoblastic T-cell lymphoma has been consistently reported to have an association with EBV. In fact, EBV-positive B-immunoblasts are detected in the lymph nodes and bone marrow in most cases of AITL.17 However, the nature of the relationship between EBV and AITL is unclear. Some authors argue that the presence of EBV reflects the profound immunodeficient state that AITL creates, while others argue that EBV itself drives the development of AITL.18,19 There have been several reports of EBV-associated B-cell proliferations in patients with AITL.20,21 In one large scale prospective series, Delfau-Larue et al. demonstrated that the presence of circulating EBV DNA is strongly correlated with the presence of circulating AITL tumor cells and that higher levels of peripheral EBV DNA at initial presentation is associated with poorer response to typical treatments.22 However, it seems that AITL has never been reported to present with an acute EBV serologic profile like that of our patient. Among the 1249 reported cases of AITL we reviewed, data on peripheral EBV DNA or serologic testing was reported in 44 cases (including 3 case series and 14 individual case reports). Among these, EBV DNA was detected in 48% (13/27), VCA-IgG was detected in 89% (31/35), EBNA-IgG was detected in 81% (26/32), but detectable EBV-IgM was not reported in a single case (0/33) (Figure 3).22-35

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