Primary Nasal CD56+ NK/T-cell Lymphoma with Cutaneous Involvement.
- Author:
Sung Eun CHANG
1
;
Ghil Suk YOON
;
Joo Ryung HUH
;
Jee Ho CHOI
;
Kyung Jeh SUNG
;
Kee Chan MOON
;
Jai Kyoung KOH
Author Information
1. Department of Dermatology, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
Nasal CD56+ NK/T cell lymphoma;
Cutaneous involvement
- MeSH:
Genes, T-Cell Receptor;
Herpesvirus 4, Human;
Humans;
Killer Cells, Natural;
Lymphocytes;
Lymphoma*;
Lymphoma, T-Cell;
Necrosis;
Neoplasm Metastasis;
Prognosis;
Skin;
T-Lymphocytes
- From:Korean Journal of Dermatology
2000;38(6):713-720
- CountryRepublic of Korea
- Language:Korean
-
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.
