Extranasal extranodal NK/T-cell lymphoma associated with systemic lupus erythematosus
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CASE REPORT
Extranasal extranodal NK/T‑cell lymphoma associated with systemic lupus erythematosus Satoshi Ichikawa1 · Noriko Fukuhara1 · Tsuyoshi Shirai2 · Tomonori Ishii2 · Ryo Ichinohasama3 · Hideo Harigae1 Received: 10 April 2020 / Revised: 7 May 2020 / Accepted: 27 May 2020 © Japanese Society of Hematology 2020
Abstract Increased incidence of lymphoproliferative disorders is reported in patients with autoimmune diseases, majority of which have a B-cell phenotype and are pathogenetically associated with the reactivation of Epstein–Barr virus (EBV). However, EBV-associated T/NK-cell lymphoma has hardly been reported. We present the case of a 68-year-old-woman, who had been diagnosed with systemic lupus erythematosus (SLE) 28 years back and was treated with various immunosuppressive agents including steroids, cyclophosphamide, and tacrolimus. She presented with a progressively worsening swelling of the right thigh for the last few months. Radiological examination revealed an intramuscular bulky tumor without any other lesions and the biopsy results led to a diagnosis of extranodal NK/T-cell lymphoma, nasal type (ENKL). Concurrent chemoradiotherapy resulted in a complete response, which has been sustained for more than 2 years without requiring additional therapy. After the initiation of chemotherapy, SLE did not worsen with the administration of low-dose corticosteroids. To the best of our knowledge, this is the first case report of a localized extranasal ENKL developing in a patient with SLE. Keywords Systemic lupus erythematosus · Extranasal extranodal NK-T-cell lymphoma · Concurrent chemoradiation
Introduction Extranodal NK/T-cell lymphoma, nasal type (ENKL) is a rare histological subtype of non-Hodgkin lymphoma. ENKL is associated with Epstein–Barr virus (EBV) infection, and the incidence is high in Asia and Central/South America where EBV infection is common. According to the results of a population-based study in a defined area in Japan, ENKL represents 1.53% of all lymphomas [1]. ENKL is resistant to anthracycline-containing chemotherapies because NK cells express P-glycoprotein which is a product of the multidrug resistance gene one [2]. Consequently, the prognosis of ENKL is generally poor [3]. The median survival time of patients with advanced stage ENKL is reported to be as short as 4 months [4]. However, ENKL localized to the nasal area * Satoshi Ichikawa [email protected] 1
Department of Hematology, Tohoku University Hospital, 1‑1 Seiryo‑cho, Sendai 980‑8574, Japan
2
Department of Rheumatology, Tohoku University Hospital, Sendai, Japan
3
Department of Hematopathology, Tohoku University Hospital, Sendai, Japan
can be well controlled by concurrent chemoradiotherapy [2]. In contrast, extranasal ENKL is reported to have a poorer prognosis [5, 6] and an optimal therapeutic strategy for it has not been yet established. Systemic lupus erythematosus (SLE) is an autoimmune disease which leads to the dysfunction of various organs and requires long-term immunosuppressive thera
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