Clinical features and treatment outcome of nasal-type NK/T-cell lymphoma of Waldeyer ring

• Published in: Blood 2008-10, Vol.112 (8), p.3057-3064
• Main Authors: Li, Ye-Xiong, Fang, Hui, Liu, Qing-Feng, Lu, Jiade, Qi, Shu-Nan, Wang, Hua, Jin, Jing, Wang, Wei-Hu, Liu, Yue-Ping, Song, Yong-Wen, Wang, Shu-Lian, Liu, Xin-Fan, Feng, Xiao-Li, Yu, Zi-Hao
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Antineoplastic Agents - therapeutic use; Biological and medical sciences; Child; Combined Modality Therapy; Disease-Free Survival; Female; Hematologic and hematopoietic diseases; Humans; Killer Cells, Natural - cytology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, T-Cell - diagnosis; Lymphoma, T-Cell - drug therapy; Lymphoma, T-Cell - radiotherapy; Male; Medical sciences; Middle Aged; Nose Neoplasms - diagnosis; Nose Neoplasms - drug therapy; Nose Neoplasms - radiotherapy; Prognosis; Treatment Outcome
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2008-05-160176

The clinical characteristics and prognosis remain unclear for nasal-type NK/T-cell lymphoma of Waldeyer ring (WR-NKTL). The aim of this study is to determine the clinical features and outcome. Ninety-one patients with WR-NKTL were reviewed. According to the Ann Arbor system, 15, 56, 12, and 8 patients had stage I, II, III, and IV. Of patients with stage I and II, 54 received combined chemotherapy and radiotherapy (CMT), 13 received radiotherapy alone, and 4 patients received chemotherapy alone. All 20 patients with stage III/IV received primary chemotherapy. The disease is characterized by predominance in young males, good performance, a propensity for nodal involvement, frequent stage II through IV diseases, low frequency of elevated LDH, low-risk international prognostic index (IPI), high sensitivity to radiotherapy, and intermediate sensitivity to chemotherapy. The 5-year overall survival and progression-free survival for all patients were 65% and 51%, respectively. The age, B symptoms, stage, and IPI were important prognostic factors. CMT tended to improve the survival compared with radiotherapy alone for patients with stage I and II diseases. Both nodal involvement and distant extranodal dissemination were the primary failure patterns. WR-NKTL appears to have distinct clinical characteristics and favorable outcomes.