Beer Timothy, Dorion Patrick
Department of General Internal Medicine, Geisinger Medical Center, Danville , PA, USA.
Department of Hematopathology, Geisinger Medical Center, Danville , PA, USA.
Hematol Rep. 2015 Jun 3;7(2):5893. doi: 10.4081/hr.2015.5893.
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.
血管免疫母细胞性T细胞淋巴瘤（AITL）是一种侵袭性外周T细胞淋巴瘤，其典型特征为就诊时出现明显的淋巴结肿大和B症状、多克隆高球蛋白血症、自身免疫性溶血以及爱泼斯坦-巴尔病毒（EBV）频繁但高度可变的累及。淋巴结活检结果通常包括淋巴结结构消失、多形性浸润、非典型T细胞（通常为CD4+/CD10+/PD1+）以及高内皮小静脉和滤泡树突状细胞的显著增殖。然而，这种经典的病理表现组合最初常常被EBV+B免疫母细胞的突出表现所掩盖，无论是否伴有外周循环EBV DNA。在此，我们记录了首例AITL患者出现急性血清学EBV特征（VCA-IgM）的报道病例，我们建议临床医生在适当的临床情况下，无论爱泼斯坦-巴尔病毒相关的检查结果如何，都要对AITL保持高度的怀疑指数。
