Nasal Type NK/T Cell Lymphoma with Cutaneous and Ocular Involvement: A Case Report.

Background: NK/T cell lymphoma is a lethal midline granuloma that produces necrotic and granulomatous lesions in the upper respiratory tract, especially in the nasal cavity. Environmental and life-style factors including infectious organisms, drugs, solvents and other chemicals together with occupation and genetical factors may play a role in the etiology of NHL. NK/T cell lymphoma is closely associated with EBV infection. This tumor affects nose and midface, but it can also arise on the skin, gastrointestinal tract, testes, CNS, lungs, salivary glands, bone marrow and larynx. 5–8% of extranodal lymphomas involve the orbit, and 1.3–2.0% of extranodal lymphomas appear as primary orbital tumors. High-grade B-cell lymphomas occasionally involve ocular adnexa, and T-cell lymphoma, NK-cell lymphoma, and Hodgkin lymphoma are rarely encountered in this site. The skin can be the primary site of NK/T cell lymphoma and the most common extranodal site when the lymphoma spreads. Aside from affecting the nasal and facial skin, the tumour can affect the skin of the postauricular area, back, trunk and upper and lower extremities. As we experienced 37-year-old woman who has nasal type NK/T cell lymphoma with cutaneous and ocular involvement, we present this case.

Case Report: A 37-year-old woman complained about right nasal obstruction, headache and rhinorrhea for 1 month. She had ocular pain, decreased visual acuity, uveitis, complete opthalmoplegia on left eye. Radiologic finding was that mass like lesion filled in right nasal sinus which invaded into maxillary sinus. Destructed septum was found between maxillary sinus and nasal sinus. Orbital CT scan was nonspecific. Abdominal and chest CT scan excluded visceral lymphadenopathy. Laboratory findings were hemoglobin 9.9g/dl, hematocrit 30.2%, platelet count 296,000/mm³, white blood count 9,100/mm³ in peripheral blood test. Bone marrow was not involved by lymphoma cell. She was treated with chemotherapy (CHOP) and we infected triamcinolone acetonede in intravitreal cavity. And then, size of mass in nasal cavity decreased. Cytologic finding was vitreous body presented with 35% atypical lymphocytes. So we choosed radiotherapy as additional treatment. After 40 days of chemotherapy, multiple erythematous to violaceous well-defined, coin sized plaques and nodules on the both extremities. After 2 days, the skin lesions rapidly disseminated to the trunk. We performed skin biopsy and observed CD3+, CD56+ immunochemistry. Her diagnosis was nasal type NK/T cell lymphoma with cutaneous and ocular involvement. And now she is treated with radiotherapy and chemotherapy (DHAP). The skin lesions improved with postinflammatory hyperpigmentation. Drooping of the left eyelid was relieved and uveitis is disappeared. But fixed pupil is not recovered. Her visual activity hasn’t got worse, although radiotherapy was treated. She should be closely observed as prognosis of NK/T cell lymphoma is usually poor. Patients with NK/T cell lymphoma have poor survival outcomes, with the cumulative probability of survival at 5 years ranging from 37.9% to 45.3%.