Atlas of Hematology

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

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

Keywords: Nazal tip NK/T-hücreli lenfoma, Radyoterapi, Peroneal sinir, Düşük ayak

Year: 2012
Number: 4
Referance No: 03360

Figures 1-3: H&E staining of the lesion located on the palate shows abundant lymphocytes.
Figure 4: The presence of EBV small RNAs in the neoplastic cells was observed via in situ hybridization using EBV-encoded small RNA (EBER) oligonucleotides.
Figure 5: Lesion on the right knee.
Figure 6: Lesion on the right arm.
Figure 7: The patient’s eyes, hyperemia, and edema.
Figure 8: Paraneoplastic lesions on the legs that ocurred during the course of chemotherapy
Figure 9: Photograph taken a few days before the patient died shows bilateral proptosis and conjunctival chemosis.
Figure 10: Swelling of the left testis.
Figure 1: Digital subtraction angiography of the hepatic artery.
Figure 2: Digital subtraction angiography of the superior mesenteric artery.

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