Rapid and fatal progression of Epstein-Bar virus-associated paracortical hyperplasia with T-cell oligoclones to overt angioimmunoblastic T-cell lymphoma: a case report

We report an unusual case of angioimmunoblastic T-cell lymphoma (AITL) progressing rapidly from Epstein-Bar virus (EBV)-associated paracortical hyperplasia with T-cell oligoclones, which caused the patient to die within only 1 month. The patient was a 67-year-old Chinese man with 3 month’s history of rhinorrhea, pruritus and superficial lymphadenopathy. In the first biopsy, morphology and immunophenotype showed reactive lymphoid hyperplasia predominated by paracortical hyperplasia. In situ hybridization of EBER and TCR gene rearrangement analysis demonstrated infection of EBV and rearrangements of TCRβ-VJ2, TCRβ-DJ and TCRγ-VJ2 genes. After about one month’s effective treatment of antiviral therapy, the patient’s condition deteriorated with extensive systemic symptoms. In the second biopsies of two involved sites, the findings of morphology, immunophenotype and genetics resulted in the diagnosis of AITL. Although received chemotherapy of COP immediately, the patient died of complications of AITL less than one month later.