Acute Epstein–Barr virus-positive cytotoxic T cell lymphoid hyperplasia in the upper aerodigestive tract, mimicking extranodal natural killer/T cell lymphoma, nasal type
To describe the clinicopathological features of nine patients with acute Epstein–Barr virus (EBV)-positive cytotoxic T cell lymphoid hyperplasia (EBV+TLH) in the upper aerodigestive tract, in which initial findings led to a preliminary misdiagnosis of extranodal NK/T cell lymphoma, nasal type (ENKTL). A series of nine cases of EBV+TLH in one Chinese institution over a 9-year interval was retrospectively analyzed. Median age was 16 years (range 5–29 years) with a M:F ratio of 5:4. All patients were previously healthy with an acute onset period of < 1 month. Six patients (66%) presented with masses or polypoid protrusions in the upper aerodigestive tract. Nasopharyngeal symptoms, cervical lymphadenopathy, and fever were found in 89%, 78%, and 56% of patients, respectively. In seven cases, morphology mainly showed small-sized irregular cells and in two cases medium-to-large cells. In all cases, the cells diffusely expressed cytoplasmic CD3 and at least one marker for cytotoxic granules, but were negative for CD56. CD5 expression was detected in eight cases (8/9, 89%). In all cases, double staining for CD3 and EBER indicated that most T cells were infected with EBV. T cell receptor gene rearrangement was performed in five cases and all showed polyclonal results. All patients achieved complete remission within 1 month after diagnosis without any chemoradiotherapy and were followed up 19–124 months without recurrent disease. EBV+TLH in the upper aerodigestive tract is occasionally observed in China. The histopathologic features of EBV+TLH can mimic ENKTL. EBV+TLH should be taken into consideration as a potential diagnosis when the disease duration is short, spontaneous remission is achieved without intervention, and when histology shows infiltration with EBV-infected T lymphocytes.
KeywordsExtranodal NK/T cell lymphoma Human herpesvirus 4 Infectious mononucleosis Upper aerodigestive tract Immunophenotyping
The authors thank Drs Sun Jirui and Ma Yin for providing clinical follow-up details, the technical staff of the immunohistochemistry laboratories at the Capital Medical University, the Beijing Friendship Hospital and the Beijing Chaoyang Hospital for performing additional double staining studies.
This work was supported by grants from the Internal Start-up Science Foundation of Beijing Friendship Hospital, China (No. Yyqdkt2013-15) and by grants from the Internal Talent Development Foundation of Beijing Chaoyang Hospital, China (No. CYMY-2017-01).
Compliance with ethical standards
All patients provided written informed consent for use of tissue samples for research and were audited by the Medical Ethics Committee of Beijing Friendship Hospital, Capital Medical University (approval number 2017-P2-129-01).
Conflict of interest
The authors declare that they have no conflicts of interest.
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