Tian Chen, Yu Yong, Zhang Yizhuo
Key Laboratory of Cancer Prevention and Therapy, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin 300060, P.R. China.
Mol Clin Oncol. 2016 Dec;5(6):777-778. doi: 10.3892/mco.2016.1042. Epub 2016 Oct 4.
Primary cutaneous extranodal natural killer/T-cell lymphoma, nasal type (ENKL-NT) is a relatively rare disease associated with aggressive tumor-cell behavior and poor prognosis. Progress in immunohistochemistry has improved the identification of ENKL-NT. The present case study reported on a 64-year-old female patient presenting with several red nodular lesions on the neck developed over four months. Cutaneous biopsy revealed these cells were positive for CD3, CD56, CD5, CD8 and negative for CD2, CD34, CD7, CD20 and Granzyme B. A computed tomography scan and bone marrow biopsy did not show any abnormalities and a diagnosis of primary cutaneous ENKL-NT was made. After treatment with chemotherapy regimens comprising cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP), followed by high-dose treatment with methotrexate, dexamethasone, ifosfamide, etoposide and L-asparaginasum (SMILE), the patient succumbed to the disease. The present study exemplified that immunohistochemical analysis as well as the recognition of atypical lymphoid cells showing angiocentricity is crucial for the correct diagnosis of ENKL-NT. The prognosis of primary cutaneous ENKL-NT remains poor. As the CHOP regimen is not completely effective, high-dose chemotherapy, such as the SMILE regimen, is required.
原发性皮肤结外自然杀伤/T细胞淋巴瘤，鼻型（ENKL-NT）是一种相对罕见的疾病，与侵袭性肿瘤细胞行为和不良预后相关。免疫组织化学的进展提高了对ENKL-NT的识别。本病例研究报告了一名64岁女性患者，其颈部出现多个红色结节性病变，病程四个月。皮肤活检显示这些细胞CD3、CD56、CD5、CD8呈阳性，CD2、CD34、CD7、CD20和颗粒酶B呈阴性。计算机断层扫描和骨髓活检未显示任何异常，遂诊断为原发性皮肤ENKL-NT。在用环磷酰胺、阿霉素、长春新碱和泼尼松（CHOP）组成的化疗方案治疗后，接着用甲氨蝶呤、地塞米松、异环磷酰胺、依托泊苷和L-天冬酰胺酶（SMILE）进行高剂量治疗，该患者最终死于该病。本研究表明，免疫组织化学分析以及识别显示血管中心性的非典型淋巴细胞对ENKL-NT的正确诊断至关重要。原发性皮肤ENKL-NT的预后仍然很差。由于CHOP方案不完全有效，需要高剂量化疗，如SMILE方案。
