A rare T-cell lymphoma in an ankylosing spondylitis patient under immunosuppression with tumor necrosis factor inhibitor

Abstract

Lymphoproliferative disorders arising in a background of immune deficiency/dysregulation correspond to a spectrum of disorders ranging from non-noxious hyperplasias to aggressive lymphomas, mainly of B-cell type. We describe a case of an Epstein-Barr virus-positive T-cell lymphoma, with a cutaneous presentation and unusual pathological features in a patient under immunosuppression with infliximab. A 60-year-old male, with a history of ankylosing spondylitis and autoimmune hemolytic anemia, treated with infliximab and low-dose prednisone since 2013, presented with a 7 cm ulcerated, well-demarcated tumor on his left lower back and a 20 cm scaly, well-demarcated erythematous patch in the left scapular region. A skin biopsy revealed a diffuse infiltration of the superficial and deep dermis by atypical, intermediate-size lymphocytes that expressed CD2, CD3, CD56, TIA-1, Granzyme B, TCRδ, and EBER. There was no evidence of epidermotropism, vasculotropism, or necrosis. The fluor-d-glucose positron emission tomography showed a large splenic, hepatic, bone marrow, and nodal uptake. A diagnosis of an extranodal NK/T-cell lymphoma in association with immunosuppression was rendered. With this article, we aim to add awareness to the difficulty of diagnosis, the careful use of immunomodulators, clinical suspiciousness, and surveillance of possible consequences warranted in all patients under prolonged immunosuppression.

Keywords

• γδ T lymphocytes. γδ T-cell lymphoma. Extranodal NK/T-cell lymphoma. Epstein-Barr virus. TNF- α inhibitors.