Extranodal NK/T cell lymphoma is a relatively uncommon type of non-Hodgkin's lymphoma, which is prevalently distributed in Asia and South America, and is highly associated with Epstein-Barr virus (EBV) infection. Due to its highly aggressive course and poor response to treatment because of its multi-drug resistance, for the timebeing there is not yet a definite treatment strategy. The clinical manifestation, pathological diagnosis and the progress of treatment methods of ENTNKCL are reviewed below.
Epstein-Barr Virus Infections ; complications ; Herpesvirus 4, Human ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; therapy ; virology

OBJECTIVETo investigate the clinical and pathological characteristics of patients with primary Waldeyer's ring lymphomas (PWRL), and to analyze its therapeutic efficacy and prognostic factors.

METHODSA total of 112 patients with PWRL confirmed by pathological and immunohistochemical methods between January 2009 and January 2014 were studied. Clinical data were collected and analyzed retrospectively.

RESULTSPWRL accounted for 3.9% of lymphoma over the same period. Median age of patients with PWRL was 51.5 years old. The affected areas were tonsil, nasopharynx, tongue base and oropharynx, which accounted for 63.4% (71/112), 22.3% (25/112), 5.3% (6/112) and 4.5% (5/112) respectively. The most common pathological types of these four areas were diffused large B-cell lymphoma (DLBCL) and extranodal NK/T cell lymphoma (NKTCL) which accounted for 58% and 15.2%. The overall response rate (CR/CRu = 51.4%; PR = 30.8%) in all patients was 82.2%, the estimated 5-year overall survival (OS) rate were 71.6%. The 5-year OS rate were 94.7% in the group used Rituximab. Meanwhile, chemotherapy combined with radiotherapy could improve the outcome of T-cell PWRL patients and the 5-year OS rate were 88.9%. Age, disease stages, pathological types, IPI scores, LDH level, β2-MG level and the efficacy of initial therapy were prognostic factors with statistical significance. Cox multivariate analysis showed that age of more than 60 years, LDH level, pathological types and the efficacy of the initial therapy were independently associated with OS.

CONCLUSIONPWRL has a relatively good prognosis. The pathological types affect the prognosis directly and guide treatment. Combined modality therapy should be chosen for patients with PWRL. Patients with T-cell PWRL should accept chemotherapy combined with radiotherapy, while rituximab may be better for B-cell PWRL. The efficacy of initial therapy is crucial for the outcome of patients. Age and LDH level are also important prognostic factors.

Combined Modality Therapy ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; pathology ; Lymphoma, Large B-Cell, Diffuse ; diagnosis ; pathology ; Middle Aged ; Multivariate Analysis ; Prognosis ; Retrospective Studies ; Rituximab ; Survival Rate

OBJECTIVE: To explore the clinical feature, the reason of misdiagnosis and mistreatment, influential factor of prognosis in patients with extranodal NK/T cell lymphoma-nasal type. METHOD: A retrospective study was made on the clinical data of 34 patients with extranodal NK/T cell lymphoma-nasal type. Among them, 10 cases were staged I(E) intra-cavity, 15 cases were I(E) ex-cavity, 6 cases were II(E) and 3 cases were IV(E). Among them, 29 cases were in nasal cavity, 5 cases were outside nasal cavity; 14 cases were treated with single chemotherapy or radiation therapy, 20 cases were treated with radiation therapy add chemotherapy. RESULT: The total rate of misdiagnosis and mistreatment were 58.8% (20/34), 52.3% (18/34), respectively. The 5-year survival rate of the I(E) intra-cavity group were 60.0% (6/10), and those of I(E) ex-cavity group were 26.7% (4/15), and those of II(E) group and IV(E) group were 16.7% (1/6), 0% (0/3), respectively There was significant difference between 3 groups by statistical analysis (P < 0.01). The 5-year survival rate of I(E) ex-cavity group treated with single therapy were 0% (0/6), and those of I(E)-cavity group treated with combined therapy were 50% (1/2). CONCLUSION The early clinical manifestation of extranodal NK/T tell lymphoma-nasal type is atypical and which is hard to diagnose and treat. Diagnosis depends on pathologic biopsy and immunohistochemistry, there are many factors that influence the prognosis of this disease, in which the clinical stage is a major factor. It is crucial for diagnosing and treating early.
Adolescent ; Adult ; Aged ; Diagnostic Errors ; Female ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; therapy ; Male ; Middle Aged ; Nose Neoplasms ; diagnosis ; therapy ; Retrospective Studies ; Survival Rate ; Young Adult

This study was purposed to explore the clinical characteristics, therapy and prognosis of patients with extranodal NK/T cell lymphoma (ENKL). 47 patients with ENKL from October 1995 to December 2008 in our hospital were analyzed retrospectively. The survival of patients was analyzed by using Kaplan-Meier methods, the prognosis of patients was evaluated by multivariate analysis using COX regression model. The clinical parameters used included CD56, Ann Arbor stage, international prognostic index (IPI) and B symptom. The results showed that the 2-year and 5-year overall survival (OS) rates were 91%, 71% respectively. Multivariant analysis by COX regression showed the CD56 and Ann Arbor stage were independent prognostic factors. Single factor analysis with staging in CHOP chemotherapy group indicated that more than stage IIIE (including IIIE) was a prognostic factor. Single factor analysis with B symptom showed that B symptom also was a prognostic factor. The cumulative survival rate of patients received radiotherapy alone was higher than that of patients got chemotherapy alone, its difference had a statistical significance, but there was no statistical significance between radiotherapy group and chemoradiotherapy group. It is concluded that CD56, Ann Arbor stage and B symptoms are prognostic factors. Radiotherapy alone is better than chemotherapy alone in therapy of ENKL. Chemotherapy combined with radiotherapy can not improve the survival. Improving the status of patients can make prognosis better.
Adolescent ; Adult ; Aged ; Chemoradiotherapy ; Female ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; therapy ; Male ; Middle Aged ; Prognosis ; Proportional Hazards Models ; Retrospective Studies ; Treatment Outcome ; Young Adult

PURPOSE: Extranodal natural killer/T-cell lymphoma, nasal type (ENKTL) is a rare subtype of non-Hodgkin lymphoma, and asparaginase-based regimens are the best first-line treatments. Data on the role of specific circulating lymphocyte subsets in the progression of ENKTL are limited. The aim of this study was to investigate the clinical correlation and distribution of circulating absolute CD4+ T-cell counts (ACD4Cs) in ENKTL. MATERIALS AND METHODS: We retrospectively searched medical records for 70 newly diagnosed ENKTL patients treated with pegaspargase-based regimens. Comparison of ACD4Cs as a continuous parameter in different groups was calculated. Univariate and multivariate analyses were used to assess prognostic factors for overall survival (OS) and progression-free survival (PFS). RESULTS: Stage III/IV, B symptoms, elevated lactate dehydrogenase, monocytopenia, high-intermediate and high risk International Prognostic Index (IPI) and Korean Prognostic Index (KPI), high risk Prognostic Index of Natural Killer Lymphoma (PINK), and lower lymphocytes were significantly associated with low ACD4C at diagnosis. With a median follow-up time of 32 months, patients who had an ACD4C < 0.30×109/L had a worse OS. Median OS was 11 months and median PFS was 5 months in the low ACD4C cohort. There were significant differences in both OS and PFS between the two cohorts. Moreover, multivariate Cox analysis identified ACD4Cs as an independent predictor for OS and PFS. CONCLUSION: Low ACD4Cs were associated with poorer survival and could act as a negative predictor for ENKTL patients treated with asparaginase-based regimens.
Cell Count* ; Cohort Studies ; Diagnosis ; Disease-Free Survival ; Drug Therapy* ; Follow-Up Studies ; Humans ; L-Lactate Dehydrogenase ; Lymphocyte Subsets ; Lymphocytes ; Lymphoma* ; Lymphoma, Extranodal NK-T-Cell ; Lymphoma, Non-Hodgkin ; Medical Records ; Multivariate Analysis ; Prognosis* ; Retrospective Studies ; T-Lymphocytes

This study was purposed to explore the therapeutic strategy and factors influencing prognosis through the analysis of clinical characteristics, genetic aberrations, treatment and prognosis of the patients with extranodal NK/T cell lymphoma (ENKTL). A total of 202 patients with ENKL from 2005-2013 were analyzed retrospectively in term of Ann Arbor stage, B symptoms, lactate dehydrogenase (LDH), international prognostic index (IPI), β2-microglobulin and the expression of MYC,HXO11,BCL-2. The survival was analyzed using Kaplan-Meier method, and the COX regression model was employed for multivariate analysis. The results showed that the 5 year overall survival (OS) rate and event-free survival (EFS) rate were 61.9% and 53.9% respectively; chemoradiotherapy was apparently better than chemotherapy alone for OS and EFS; OS and EFS of the patients with BCL-2 expression were apparently better than patients without BCL-2 expression; multivariant analysis by COX regression showed that Ann Arbor stage and BCL-2 expression were independent prognostic factors for EFS/OS. It is concluded that chemoradiotherapy is better than chemotherapy alone for treatment of ENKL. Ann Arbor stage and BCL-2 expression are independent prognostic factors.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Disease-Free Survival ; Female ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; therapy ; Male ; Middle Aged ; Multivariate Analysis ; Prognosis ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Retrospective Studies ; Survival Rate ; Treatment Outcome ; Young Adult

CD4 Antigens ; metabolism ; CD56 Antigen ; metabolism ; Dendritic Cells ; pathology ; Diagnosis, Differential ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Hematologic Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Leukemia, Myeloid ; pathology ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Skin Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery

Adolescent ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cisplatin ; administration & dosage ; Dexamethasone ; administration & dosage ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; pathology ; Lymphoma, T-Cell, Cutaneous ; drug therapy ; pathology ; Lymphomatoid Granulomatosis ; pathology ; Male ; Middle Aged ; Natural Killer T-Cells ; pathology ; Neoplasm Recurrence, Local ; Skin Neoplasms ; drug therapy ; pathology ; Young Adult

Adult ; CD56 Antigen ; metabolism ; Combined Modality Therapy ; Diagnosis, Differential ; Humans ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; therapy ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Neprilysin ; metabolism ; Proto-Oncogene Proteins c-bcl-6 ; metabolism

Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; CD4 Antigens ; metabolism ; CD56 Antigen ; metabolism ; Cyclophosphamide ; therapeutic use ; Dendritic Cells ; pathology ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Humans ; Leukemia, Myeloid ; metabolism ; pathology ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Plasmacytoma ; drug therapy ; metabolism ; pathology ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; metabolism ; pathology ; Prednisone ; therapeutic use ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; Vincristine ; therapeutic use