Treatment of relapsed extranodal natural killer/T-cell lymphoma with bortezomib plus fludarabine

Extranodal NK/T cell lymphoma (ENKL) is a rare entity, associated with an aggressive clinical behavior and poor prognosis. The optimal treatment strategies, particularly for relapsed patients, have not been clearly determined. A 40-year-old female ENKL patient with orbital involvement as the first presentation was initially suspected to have an orbital pseudotumor. When the patient developed disease progression following treatment with methylprednisolone, biopsy of the orbital mass was performed. Histopathological examination of the biopsy specimen was consistent with the diagnosis of ENKL (Ann Arbor stage IE). The patient was then treated with radiotherapy followed by chemotherapy with dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide (SMILE regimen). Despite an initial good response, the patient developed disease relapse and progression (stage IIIE). Subsequently, treatment was switched to a novel regimen comprising bortezomib and fludarabine as salvage therapy for two courses. A positron emission tomography scan revealed disappearance of the orbital tumor. The patient then received autologous hematopoietic stem cell transplantation. At the last follow-up (March 2017), the patient remained disease-free.