Differential diagnosis of Wegener's granulomatosis and extranodal NK/T-cell lymphoma, nasal type

Abstract

Men aged over 40 years more commonly develop NK/T-cell lymphomas (NK/T-CL). The paper describes a case of NK/T-CL in a 20-year-old man. Despite the fact that the disease (nasal septum perforation, hard palate bone destruction, recurrent nasopharyngeal bleeding, considerable weight loss, and high erythrocyte sedimentation rate,) progressed rapidly for 5 months, the patient was found to be diagnosed as having Wegener' granulomatosis (WG). Repeated incisional biopsies showed massive necrotic changes with no clear histological verification of the diagnosis. The absence of lung and kidney lesions typical of WG, the lack of antineutrophil antibodies, and the detection of Epstein-Barr virus DNA in blood could presume NK/T-CL and confirm it by extended biopsy to have materials sufficient for morphological and immunomorphological studies. This observation shows that the disease may occur at a young age and rapidly progress; only early diagnosis can improve prognosis in patients with this type of lymphomas.

Keywords

• natural killer nk-cells
• nk/t cell lymphoma
• nasal type
• aggressive nk-cell leukemia
• median lethal granuloma
• immunoblastic t-cell lymphoma
• wegener's granulomatosis
• vasculitis necroticans in systemic lupus erythematosus and sjögren's diseas