Turkish Journal of Hematology (Dec 2012)

Nasal Natural Killer/T-cell Lymphoma with Skin, Eye, and Peroneal Nerve Involvement

  • Burcu Türker,
  • Burak Uz,
  • Metin Işık,
  • Özlen Bektaş,
  • Haluk Demiroğlu,
  • Nilgün Sayınalp,
  • Aysegül Üner,
  • Osman İlhami Özcebe

DOI
https://doi.org/10.5505/tjh.2012.03360
Journal volume & issue
Vol. 29, no. 4
pp. 413 – 419

Abstract

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Nasal-type natural killer (NK)/T-cell lymphoma (NKTL) is a rare disease strongly associated with Epstein-Barr virus and is often localized to the upper aerodigestive tract at presentation. Extranodal NKTL may involve any extranodal site and disease beyond the nasal cavity is highly aggressive, with short survival time and poor response to therapy. Herein we present a 57-year-old male that had been treated with systemic chemotherapy and cranial radiotherapy for nasaltype NKTL in the palate with skin, right eye, and right peroneal nerve involvement. He was given salvage chemotherapy consisting of 3 cycles of ICE and his response to the therapy was satisfactory, except for persistent right drop foot. About 6 weeks later, the patient presented with bilateral total loss of vision and proptosis; therefore, DHAP chemotherapy was started. Unfortunately, after 1 cycle of the second salvage chemotherapy, he died due to severe fungal infection of the hard palate. Despite the fact that involvement of any extranodal site is possible, concurrent involvement of many systems in NKTL patients is unusual. Nasal-type NKTL has a poor prognosis, despite local radiotherapy and systemic chemotherapy. Physicians should be aware of this rare disorder than can only be diagnosed after extensive immunohistochemical studies.

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