Primary Nasal CD56+ NK/T-cell Lymphoma with Cutaneous Involvement

Chang, Sung Eun; Yoon, Ghil Suk; Huh, Joo Ryung; Choi, Jee Ho; Sung, Kyung Jeh; Moon, Kee Chan; Koh, Jai Kyoung

Abstract

BACKGROUNDS: There are recently increasing reported cases of patients with malignant lymphoma with unique characteristics, designated nasal-type T/natural killer (NK) cell lymphoma (NKTL), which usually expresses the NK cell marker and shows frequent extra-nodal involvement including skin and poor prognosis. The CD56+ NKTL can be divided into nasal and non-nasal types. Nasal and non-nasal (nasal type) CD56+ NKTL share the same pathologic features such as angiocentricity and necrosis. OBJECTIVE: We have studied clinicopathologic features of 5 cases of nasal CD56+ NKTL with cutaneous involvement to further elucidate the behaviour of nasal CD56+ NKTL. RESULTS: Clinically, the cutaneous lesions were variable. Four of the five cases died with rapidly progressive disease within 25 months. Histologically, variable sized pleomorphic lymphoid cells with or without prominent angiodestruction were observed. Severe necrosis was consistent features. The results of immunophenotypes(both CD56+ and CD3+) and TCR gene rearrangement study showed these 5 cases are of NK cell origin(4 cases) or NK-like T-cell origin (1 case). A firm association with Epstein-Barr virus (EBV) infection was confirmed in our study. CONCLUSION: Although the prognosis of nasal CD56+ NKTL was not so bad, higher stage of nasal CD56+ NKTL with cutaneous metastasis seemed to be very fatal. The term NKTL has to be further specified according to immunophenotypes such as CD56+ or true T-cell, NK-like T cell lymphoma since biologic behaviour of each subgroup can be different.