Midline NK/T-cell lymphoma nasal-type: treatment outcome, the effect of L-asparaginase based regimen, and prognostic factors

PURPOSE: Midline NK/T-cell lymphoma nasal-type is an aggressive neoplasm with poor prognosis in most instances. To improve the treatment outcome, we have tried using L-asparaginase based regimen as salvage regimen plus radiation in CHOP failures, and report here the therapeutic results and prognostic factors. PATIENTS AND METHODS: From March 1992 to January 2005, 46 Chinese patients with midline NK/T-cell lymphoma nasal-type were initially treated with CHOP regimen as first-line chemotherapy. The patients who failed CHOP regimens were altered to receive L-asparaginase based salvage regimen. All the patients received primary involved-field radiation after chemotherapy. RESULTS: Thirteen patients (28.3%) sensitive to CHOP regimen received CHOP regimen for 6 cycles plus locoregional radiation, and achieved complete remission (CR). Thirty-three patients failed CHOP regimen were altered to received L-asparaginase-based salvage regimen for 2 approximately 6 cycles (median 3 cycles) plus locoregional radiation, and seventeen of the 33 CHOP failures (51.5 %) (L-asparaginase group) reached CR. The CR rate for the whole group was 65.2% (30/46 cases). The 5-year overall survival (OS) rates were 64.5% for the whole group and 55.9% for L-asparaginase group, respectively. On univariate analysis, disease stage, fever symptom and performance status were significant factors influencing overall survival. On multivariate analysis, only disease stage and fever symptom remained as independently significant factors influencing OS. CONCLUSION: In this preliminary study, the results indicated that L-asparaginase based regimen might be a promising new salvage chemotherapeutic regimen in CHOP failures and conduce to improve the treatment outcome in midline NK/T-cell lymphoma, nasal-type.     

Nasal-type NK/T cell lymphoma: clinical features and treatment outcome

Nasal-type NK/T cell lymphoma is an increasingly recognised disease entity of aggressive clinical behaviour. The objective of this study was to investigate clinical features and treatment outcomes in patients with nasal-type NK/T cell lymphoma. From January 1991 to December 2003, 26 patients diagnosed as nasal-type NK/T cell lymphoma were included in the analysis. One half of patients presented with poor performance status (ECOG > or =2); 46% of patients were categorised as high intermediate or high-risk group according to IPI; and 46% of patients were diagnosed at advanced stage. The median survival for 26 patients with nasal-type NK/T cell lymphoma was 7.4 months (95% CI, 0.1, 16.9). The treatment outcome of primary anthracycline-based chemotherapy was poor: 60% CR rate in localised disease and 0% CR rate in advanced disease. After a median follow-up of 24.4 months (range 3.1-99.0) in patients with localised disease who had achieved a CR (range 29.6-165.7), three patients (50.0%) developed disease recurrence at 6.1, 21.8, and 52.1 months, respectively, and all patients presented with locoregional failure. The predictive factors for poor survival were of age greater than 60, advanced stage and poor performance in multivariate analysis. In conclusion, Nasal-type NK/T cell lymphomas showed a poor response to the conventional anthracycline-based chemotherapy, and thus an investigation for an innovative therapy is urgently needed to improve survival in these patients.     

Lack of correlation between P-glycoprotein and chemotherapy resistance in nasal NK/T-cell lymphomas

Thirty patients with nasal natural killer (NK)/T-cell lymphoma, who underwent systemic chemotherapy with or without involved-field radiotherapy between 1993 and 1998, were retrospectively reviewed to determine the clinical significance of P-glycoprotein immunohistochemically identified in tumor specimens. Eighty percent of previously untreated patients expressed P-glycoprotein. According to P-glycoprotein immunoreactivity, all patients with nasal NK/T-cell lymphoma were divided into 2 groups; (a) P-glycoprotein-negative group (N = 6) and (b) P-glycoprotein-positive group (N = 24). There was no significant difference in clinical profiles between both groups. Regardless of the P-glycoprotein expressions, Epstein-Barr virus genomes were almost identically detected in patients of the 2 groups. Contrary to our expectations, however, P-glycoprotein expressions were not found to be a strong predictor of chemotherapy resistance. Although 2 (33%) of 6 P-glycoprotein-negative patients and 10 (42%) of the 24 P-glycoprotein-positive patients showed a favorable response to systemic chemotherapy, 4 (67%) of 6 P-glycoprotein-negative patients did not achieve complete response (CR) to chemotherapy, which led to an early death, whereas 4 (17%) of the 24 P-glycoprotein-positive patients achieved CR to chemotherapy despite positive P-glycoprotein immunoreactivity. Overall, there were no significant differences in either CR rate or the response rate of patients in the two groups. Overall 5-year actuarial survival and disease-free survival for all patients were 44% and 47%, respectively, but no differences in survival rates were observed between 2 groups. (5-year actuarial survival rate: 33% for the P-glycoprotein-negative, 50% for the P-glycoprotein-positive) (P = 0.7093, log-rank). On univariate and multivariate analyses, P-glycoprotein expressions by immunohistochemical study were not found to be an important prognostic factor. Given these observations, we conclude that the molecular mechanisms of resistance to chemotherapy in nasal NK/T-cell lymphoma patients are not entirely dependent on P-glycoprotein, and that other complex mechanisms of drug action and resistance may be likely to be involved.     

37例鼻腔NK/T细胞淋巴瘤临床分析

目的分析鼻腔NK/T细胞淋巴瘤的临床特征、不同治疗方法的疗效及影响预后的因素。方法回顾性分析1999年12月至2008年4月收治的37例经病理证实的鼻腔NK/T细胞淋巴瘤患者的临床资料。根据AnnArbor分期,Ⅰ期17例,Ⅱ期15例,Ⅲ期1例,Ⅳ期4例。单纯放疗8例,单纯化疗9例,其余20例采用放化疗联合治疗。单因素分析采用Kap lan-M e ier法,多因素分析运用Cox比例风险模型。结果全组中位生存时间27月,3年总生存（OS）率为50.0%,放化疗联合、单纯放疗、单纯化疗3年生存率分别为77.8%、50.0%、0%。单纯放疗、放化疗联合治疗后3年生存率明显高于单纯化疗（P〈0.01）;放化疗联合与单纯放疗治疗后3年生存率无明显差异（P〉0.05）。单纯化疗CR 1例（11.1%）,单纯放疗CR 6例（75.0%）,放化疗联合CR 16例（80.0%）,单纯放疗、放化疗联合治疗后CR率明显高于单纯化疗（P〈0.01）;放化疗联合与单纯放疗治疗后CR率差异无统计学意义（P〉0.05）。单因素分析显示,PS评分、IPI、LDH水平、临床分期、治疗模式、首程治疗后CR率等与预后相关。多因素分析显示,首程治疗后CR率、PS评分是鼻腔NK/T细胞淋巴瘤预后不良的独立因素。结论鼻腔NK/T细胞淋巴瘤单纯放疗、放化疗联合近期疗效显著优于常规化疗,化疗加入放疗并未改善生存率。首程治疗后CR率、PS评分可作为判断鼻腔NK/T细胞淋巴瘤预后的参考因素。     

早期原发鼻腔与韦氏环NK/T细胞淋巴瘤临床特征和预后比较

目的 探讨Ⅰ_E~Ⅱ_E期原发鼻腔与原发韦氏环NK/T细胞淋巴瘤在临床特征和预后等方面的差别。方法 回顾分析1991-2011年本院初治的273例患者,其中Ⅰ_E期184例,Ⅱ_E期89例。原发鼻腔(NC-NKTL)209例和原发韦氏环(WR-NKTL)64例。258例(94.5%)患者先接受化疗,多数患者接受CHOP或类似方案化疗,放疗中位剂量为54 Gy。结果 与NC-NKTL相比,WR-NKTL中Ⅱ_E期和有B症状者显著增多。两组治疗后有效率相近(88.7%和87.9%,P=0.869)。随访率96.3%,随访时间满5年者196例。5年总生存率(OS)和无进展生存率(PFS)分别为52.6%和41.4%,其中NC-NKTL的5年OS有高于WR-NKTL的趋势(57.0%∶39.0%,P=0.062),而5年PFS则高于WR-NKTL的(46.7%∶25.8%,P=0.019)。结论 早期原发韦氏环NK/T细胞淋巴瘤较原发鼻腔病变更易发生全身症状和颈部淋巴结转移,预后较差,临床上考虑提早放疗和颈部淋巴结预防照射。     

96例外周T细胞非霍奇金淋巴瘤预后分析

目的：分析外周T细胞淋巴瘤的临床特征、近期疗效、远期生存及预后因素。方法：对96例患者的临床特征、治疗效果及预后因素进行分析。外周T细胞淋巴瘤-非特异型（PTCL-U）66例,间变大细胞性淋巴瘤（ALCL）6例,NK/T淋巴廇（NK/TCL）17例,肠道T细胞性淋巴瘤（ITCL）5例,血管免疫母T细胞性淋巴瘤（AITCL）2例。结果：96例患者中89例接受CHOP方案为主的联合化疗,有效率（RR）为88.8%,完全缓解（CR）率为57.3%。中位随访时间30（2～98）个月,死亡52例,中位生存期31.9个月,1、3、5年生存率分别为83.3%、42.7%、35.1%。多因素分析结果显示,IPI评分是PTCL的独立预后指标（P〈0.05）。结论：外周T细胞淋巴瘤常规化疗近期疗效尚可,但远期生存率低,预后不良,需进一步探索新的治疗策略。     

Clinical significance of cyclooxygenase-2 expression in extranodal natural killer (NK)/T-cell lymphoma, nasal type

PURPOSE: To determine whether there are any differences in therapeutic response, patterns of systemic recurrence, and prognosis of patients with extranodal natural killer (NK)/T-cell lymphoma, nasal type, by the cyclooxygenase-2 (COX-2) expression. PATIENTS AND METHODS: Thirty-four patients with Ann Arbor Stage I and II extranodal NK/T-cell lymphoma who underwent chemotherapy or radiotherapy, or both, were retrospectively reviewed. These patients were divided into two groups according to their immunohistochemical staining for COX-2 expressions: a COX-2-negative group (n = 10 patients) and a COX-2-positive group (n = 24 patients). The treatment response, patterns of treatment failure, and survival data for the patients were compared between the COX-2-positive and negative groups. RESULTS: There was no significant difference in the clinical profiles between the COX-2-negative and COX-2-positive groups. All patients (100%) in the COX-2-negative group achieved complete response after initial treatment, whereas only 14 patients (58%) in the COX-2-positive group achieved complete response (p = 0.03). Compared with the patients in the COX-2-negative group, those in the COX-2-positive group had a significantly lower 2-year systemic recurrence-free survival rate (100% for the COX-2-negative group vs. 54% for the COX-2-positive group) (p = 0.02) and a decreased 5-year overall survival rate (70% for the COX-2-negative group vs. 32% for the COX-2-positive group) (p = 0.06). CONCLUSION: Cyclooxygenase-2 expression can serve as a predictive factor for poor treatment response, higher systemic recurrence, and unfavorable prognosis in patients with extranodal NK/T-cell lymphoma, nasal type.     

Failure patterns and clinical implications in early stage nasal natural killer/T-cell lymphoma treated with primary radiotherapy

BACKGROUND:

This study aimed to evaluate the failure patterns and clinical implications in patients with early stage nasal natural killer (NK)/T‐cell lymphoma treated with primary radiotherapy.

METHODS:

Two‐hundred fourteen patients were included. There were 182 cases of stage I and 32 cases of stage II disease. Patients received radiotherapy alone (n = 96) or radiotherapy and chemotherapy (n = 118). The median dose was 50 grays, and most patients received doxorubicin‐based chemotherapy.

RESULTS:

The 5‐year overall survival (OS) and progression‐free survival rates for all patients were 72% and 65%, respectively. Sixty‐three patients experienced treatment failure. The 5‐year cumulative incidences of locoregional, systemic, and overall failures were 12.0%, 25.5%, and 32.9%, respectively. Stage and paranasal extension were significant predictors for systemic failure. The 5‐year cumulative incidence of systemic failure was 22.6% for stage I disease versus 42.7% for stage II disease (P < .001), and 16.9% for limited disease versus 30.4% for paranasal extension (P < .001), respectively. Adding chemotherapy to extended involved‐field radiotherapy did not significantly decrease the systemic failure rate nor improve survival. The cumulative incidence of systemic failure and OS rate at 5 years were 24.1% and 74.4% for combined modality therapy compared with 28.5% (P = 0.758) and 69.8% (P = 0.529) for radiotherapy alone. A very low incidence of cervical lymph node or central nervous system relapse was observed.

CONCLUSIONS:

Patients with early stage nasal NK/T‐cell lymphoma have excellent locoregional control and favorable prognosis with radiotherapy, but patients with stage II disease or paranasal extension are at high risk of systemic failure, emphasizing the importance of integration of optimal radiotherapy with innovative systemic therapy. Cancer 2011;. © 2011 American Cancer Society.     

Treatment outcome of angiocentric T-cell and NK/T-cell lymphoma, nasal type: radiotherapy versus chemoradiotherapy

OBJECTIVE: The purpose of this study was to evaluate the treatment outcome of angiocentric T-cell and natural killer (NK)/T-cell lymphoma, nasal type. METHODS: Between February 1989 and March 2001, 53 patients with newly diagnosed angiocentric T-cell and NK/T-cell lymphoma, nasal type involving the head and neck, were treated with radiation therapy (RT). There were 37 males and 16 females. The median age of the patients was 45 years (range 19-73). Twenty of them were treated with chemoradiotherapy (CRT), while 33 with treated with RT alone. The median follow-up period was 74 months (range 6-173). RESULTS: The 5-year overall survival rate of all patients was 69%. CRT appeared to be inferior to RT alone in terms of 5-year overall survival, though the difference was not statistically significant (59 versus 76%, P = 0.27). CONCLUSIONS: There was no difference in survival between RT and CRT in angiocentric T-cell and NK/T-cell lymphoma, nasal type.     

Angiocentric T-cell and NK/T-cell lymphomas: radiotherapeutic viewpoints

PURPOSE: To investigate the patterns of local failure and the risk factors predictive of local failure and to establish the dose-response relationships influencing the probability of local control in patients with Stage I and II angiocentric T-cell or natural killer (NK)/T-cell lymphoma who were treated with radiotherapy (RT) alone. METHODS AND MATERIALS: We retrospectively reviewed the data from 102 patients with Ann Arbor Stage I and II angiocentric T-cell or NK/T-cell lymphoma who underwent RT alone to a median dose of 45 Gy (range, 20-70 Gy) between 1976 and 1998. The patterns of local failure, risk factors predictive of local failure, dose-response relationships, and survival data were analyzed. Because of the protean feature of local recurrences, the sites of local failure were allocated to one of three categories: true recurrence (TR), marginal recurrence (MR), and elsewhere recurrence (ER). RESULTS: Despite a higher complete remission rate (72%) after RT, 60 patients experienced treatment failure, including local failure in 48 (47%), regional failure in 3 (3%), and systemic failure in 28 (27%). The patterns of local failure were TR in 42, MR in 3, and ER in 5 patients. The median time to recurrence for TR/MR was shorter than that for ER (1 month for TR/MR vs. 12 months for ER). Patients with TR/MR had a more unfavorable prognosis than those experiencing ER (2-year survival rate after salvage treatment: 6% for TR/MR vs. 80% for ER; p <0.01). The dose-response curve was sigmoid in shape within the range of 20-54 Gy, which followed the plateau at doses in excess of about 54 Gy. A positive correlation was observed in the dose-response curve for the probability of local control (p = 0.017, logistic regression analysis). The overall 5-year actuarial survival and local recurrence-free survival rate for all patients was 42% and 53%, respectively. Achievement of complete remission was the most statistically significant risk factor predictive of TR/MR and the most important prognostic factor. CONCLUSION: Our data confirm that local failure remains the major obstacle for patients who receive RT alone and that achievement of complete remission is a particularly important determinant of treatment success. Although dose escalation up to >54 Gy cannot entirely reduce the incidence of TR/MR, we believe it is important to identify an appropriate subset of patients for whom an additional boost dose may be beneficial. Given the high rate of local failure, an investigational approach should be conducted to supplement RT using radiosensitizers or more effective chemotherapeutic agents in future trials.     

A novel prognostic model for extranodal natural killer/T-cell lymphoma

Natural killer (NK)/T-cell lymphoma is a rare neoplasm with heterogeneous clinical behaviors. This study aimed to devise a prognostic model specifically for extranodal NK/T-cell lymphoma, providing risk stratification in affected patients. A total of 146 patients newly diagnosed with extranodal NK/T-cell lymphoma were retrospectively analyzed. Independent predictors of survival were determined by Cox regression analysis. The estimated 5-year overall survival rate for all patients was 41.9%. Both the International Prognostic Index and Korean NK/T-cell lymphoma Prognostic Index were prognostic in univariate analysis. The majority of our patients were in the low-risk International Prognostic Index and Korean NK/T-cell lymphoma Prognostic Index category (with no or one adverse factor), but both of these two prognostic models had no discriminating power within the subgroup of these patients. Absolute lymphocyte count at diagnosis, B symptoms, and advanced stage were independently predicted poorer survival. Cox analysis yielded a novel prognostic model based on these three parameters that categorized patients into one of three risk groups: Group 1 (57 cases, 39.0%), no adverse factors; Group 2 (43 cases, 29.5%), one adverse factor; and Group 3 (46 cases, 31.5%), two or three adverse factors. Five-year overall survival was 71.6% for Group 1, 55.5% for Group 2, and 0% for Group 3 (P < 0.0001). The novel prognostic model balanced the distribution of patients into different risk groups with better predictive discrimination. It may be useful to stratify patients with extranodal NK/T-cell lymphoma into different risk groups and provide more information for treatment selection.     

含吉西他滨方案和CHOP样方案一线治疗结外NK/T细胞淋巴瘤效果观察

目的 比较含吉西他滨的联合方案和CHOP样联合方案一线治疗结外NK/T细胞淋巴瘤的效果及安全性.方法 回顾性分析河南省人民医院2012年3月至2017年3月39例初治结外NK/T细胞淋巴瘤患者,其中11例采用含吉西他滨联合方案,28例采用CHOP样联合方案.比较两组的完全缓解(CR)率、部分缓解(PR)率、总反应率(ORR)、总生存(OS)率、无进展生存(PFS)率及不良反应.结果吉西他滨组CR 5例,PR 3例;CHOP样组CR 8例,PR 5例;两组CR率和ORR比较,差异均无统计学意义(P=0.453,P=0.073);两组预计3年OS率(75%比33%)、3年PFS率(70%比29%)比较,差异均有统计学意义(χ2=5.606,P=0.018;χ2=3.924,P=0.048).单因素分析结果显示,治疗方案(P=0.018)、乳酸脱氢酶(LDH)升高(P=0.007)影响患者生存预后(均P<0.05).多因素分析结果显示,LDH升高增加患者的死亡风险(RR=6.331,95%CI2.339~17.136,P<0.001),采用含吉西他滨方案治疗降低患者的死亡风险(RR=0.101,95%CI0.023~0.452,P=0.003).不良反应多为1~2级,患者耐受性良好.结论 含吉西他滨联合方案较CHOP样联合方案可延长结外NK/T细胞淋巴瘤患者的生存时间,提高远期疗效.     

Major prognostic factors of adult patients with advanced T-cell lymphoma/leukemia

Eighty-one adult patients with advanced T-cell lymphoma/leukemia including 54 with adult T-cell leukemia/lymphoma (ATL), who were treated between 1981 and 1983 with vincristine, cyclophosphamide, prednisolone, and doxorubicin (VEPA) or VEPA plus methotrexate (VEPA-M) in randomized fashion, were evaluated for pretreatment characteristics. The overall complete response (CR) and the 4-year survival rates were 39.5% and 19.4%, respectively, and 69% of 32 CR patients had relapses, indicating the need for development of new effective regimens for the disease. In a multiple logistic regression analysis, only three factors, leukemic manifestation, poor performance status (PS), and a high lactate dehydrogenase (LDH) level, were significantly associated with the poor response rate. In a Cox proportional hazards model analysis, shortened survival was again significantly associated with poor PS and a high LDH level, but not with a clinical diagnosis of ATL. The two factors, PS and LDH level, that were found to be significantly associated with both CR and survival rates, were used to construct a model containing six categories of patients at increasing risk for poor response and shortened survival. These categories divided the patients into three groups with respective CR and 4-year survival rates of 75% and 53% for low-risk, 45% and 15% for moderate-risk, and 15% and 0% for high-risk. The results indicate that PS and LDH levels were the most important in predicting the response and survival of an adult patient with advanced T-cell lymphoma/leukemia. The prognosis of patients with usual peripheral T-cell lymphoma, excluding ATL, was comparable with that of advanced B-cell lymphoma. These results have important implications for the design of new prospective therapeutic trials.     

IMVP-16/Pd followed by high-dose chemotherapy and autologous stem cell transplantation as a salvage therapy for refractory or relapsed peripheral T-cell lymphomas

The present study aimed to analyse the treatment outcome of IMVP-16/Pd (ifosfamide, methotrexate, etoposide and prednisone) followed by high-dose chemotherapy and autologous stem cell transplantation (HDC/ASCT) for patients with peripheral T-cell lymphomas (PTCLs) who were previously treated with CHOP. Since 1995, 32 PTCL patients were treated with IMPV-16/Pd. Nine of 32 patients achieved a response (5 demonstrating complete response (CR) and 4 partial response), with an overall response rate of 28.1% (95% onfidence interval 0.12-0.45). Considering histopathologic subtypes, 3 of 4 relapsed natural killer (NK)/T-cell lymphoma patients (75%) achieved CR, but only 1 of 6 in non-NK/T-cell lymphoma patients (16.7%) achieved CR (P = 0.19). Six of 9 IMVP-16/Pd sensitive patients underwent HDC/ASCT. Three of them relapsed after 3, 4 and 15 months, respectively, of HDC/ASCT. Estimated 3-year overall survival and progression-free survival rates were 14.2% and 12.2%, respectively. Multivariate analysis revealed that responsiveness to first-line CHOP was a significant prognostic factor (P < 0.05). These results indicate that IMVP-16/Pd followed by HDC/ASCT appears to be an effective salvage regimen, especially for NK/T-cell lymphoma.     

Early stage nasal NK/T-cell lymphoma: clinical outcome,prognostic factors,and the effect of treatment modality

PURPOSE: To determine the clinical outcome, prognostic factors, and effect of adding combination chemotherapy to radiation therapy on disease control and survival in early stage nasal natural killer (NK)/T-cell lymphoma. METHODS AND MATERIALS: A retrospective "intent to treat" analysis was carried out on 79 patients treated consecutively with curative intent between 1977 and June 2001. They all had early stage (Ann Arbor Stage I(E): 63, II(E):16) nasal NK/T-cell lymphoma. Sixty-one were planned for combined modality treatment (CMT); radiotherapy alone (RT) was intended for 18. Three to 6 cycles of anthracycline-containing regimens were aimed at for patients intended for CMT. Patients selected for RT were generally older or treated during the earlier part of the study period. RESULTS: The overall complete response (CR) rate was 68.4% (54/79), of whom 44.4% (24/54) relapsed after 54.9 months median follow-up of the survivors. The 5-year disease-free survival (DFS) and overall survival (OS) rates were 35.5% and 37.9%, respectively. On multivariate analysis, good performance status (Eastern Cooperative Oncology Group [ECOG] <2) was shown to be a significant favorable factor for DFS (p = 0.011), whereas good performance status (ECOG <2) and Ann Arbor Stage I(E) disease were shown to be significant favorable factors for OS (p = 0.001 and p = 0.013, respectively). The type of intended treatment was not a significant factor for DFS (5-year DFS CMT vs. RT = 35.8% vs. 30.5%, p = 0.795) or OS (5-year OS CMT vs. RT = 40.3% vs. 29.8%, p = 0.693) though only 2 of the 16 Stage II(E) patients were intended for RT alone. Resistance to treatment, especially to chemotherapy, was common. Of 61 patients intended to be given CMT, 31 showed disease progression while receiving chemotherapy, of whom 17 progressed locoregionally. Nine of the latter group were rendered CR by salvage radiotherapy. CONCLUSIONS: The overall outcome in early stage nasal NK/T-cell lymphoma is poor. Performance status and Ann Arbor stage are significant factors influencing DFS and OS. The addition of anthracycline-containing chemotherapy to radiotherapy does not appear to confer any survival benefit in Stage I(E) patients. Therefore, radiation therapy remains the mainstay of treatment for this lymphoma type.     

A large single-center experience with allogeneic stem-cell transplantation for peripheral T-cell non-Hodgkin lymphoma and advanced mycosis fungoides/Sezary syndrome

BackgroundThe prognosis for patients with most forms of T-cell lymphoma is poor. Allogeneic hematopoietic stem-cell transplantation (HSCT) may improve the outcome.Patients and methodsThis study examines the outcome of 52 patients who underwent ablative or nonablative allogeneic HSCT for peripheral T-cell lymphoma (PTCL) or advanced mycosis fungoides/Sezary syndrome over a 12-year period at a single institution. We divided the patients into those with predominantly nodal histologies: peripheral T-cell not otherwise specified (PTCL NOS), angioimmunoblastic (AITL), or anaplastic large cell lymphoma, T/null type (systemic) (ALCL), and predominantly extranodal histologies: natural killer (NK)/T cell, enteropathy type, hepatosplenic, subcutaneous panniculitic, mycosis fungoides, or T cell or NK cell other.ResultsMedian follow-up of survivors is 49 months. Non-relapse mortality and relapse at 3 years was 27% and 43%, respectively. The incidence of grade II–IV acute graft-versus-host disease (GVHD) was 21%. The incidence of extensive chronic GVHD at 2 years was 27%. The 3-year progression-free survival was 30%: 45% in patients with predominantly nodal histologies (PTCL NOS, AITL, and ALCL) and 6% in patients with predominantly extranodal histologies (P = 0.016). Overall survival at 3 years was 41% for all patients.ConclusionAllogeneic HSCT can produce long-term remissions in relapsed/refractory T-cell lymphoma, especially those with nodal histologies.     

Clinical analysis of patients with primary and secondary extranodal natural killer/T-cell lymphoma of central nervous system

Extranodal natural killer (NK)/T-cell lymphoma (NKTL) is a rare non-Hodgkin lymphoma that rarely arise exclusively in or metastasizes to the central nervous system (CNS). Globally, CNS involvement of NKTL heralds a serious prognosis and there is no standard treatment. 19 of 414 patients (4.59%) with ENKL followed were diagnosed with CNS involvement between 2006 and 2020. Two patients had primary CNS (PCNS) NKTL, and 17 patients had secondary CNS (SCNS) invasion. A total of 9 patients survived and 10 patients died. The median overall survival time was 55 months, and the median survival time after CNS invasion was 17 months. The 5-year cumulative survival probability was 45.7%. In conclusion, CNS risk evaluation and prophylaxis treatment can be carried out for patients with NK/T-cell lymphoma prognostic index risk group III/IV. In terms of treatment, systemic therapy based on methotrexate combined with radiotherapy and intrathecal chemotherapy can be selected.     

鼻腔自然杀伤/T细胞淋巴瘤放射治疗的疗效和影响因素

 目的　回顾分析鼻腔自然杀伤（NK）／T细胞淋巴瘤的放射治疗效果,并分析其预后因素。方法　回顾分析9年间接受放射治疗的62例鼻腔NK／T细胞淋巴瘤的临床资料和疗效,单因素分析采用Kaplan-Meier法,多因素分析用COX比例风险模型。结果　全组中位生存时间69.7个月（95 % CI为63.0～78.0个月）,3、5年总生存率分别为66.1 %和46.8 %,远处转移导致治疗失败占61.8 %。T淋巴细胞CD3升高组和降低组的中位生存时间分别为72.6个月和39.6个月,两组比较差异有统计学意义（χ2＝4.9309,P＝0.0264）。多因素分析表明,修正后国际预后指数（IPI）为0～1（χ2＝7.5266,P＝0.0061）、CD3升高（χ2＝9.0912,P＝0.0266）和治疗结束达到CR（χ2＝9.0912,P＝0.0106）是影响鼻腔NK／T细胞淋巴瘤放疗总生存的有利预后因素。结论　放射治疗鼻腔NK／T细胞淋巴瘤疗效肯定,但远处转移治疗失败率高,全身治疗仍具有重要地位;修正后IPI为0～1、CD3升高、治疗结束达到CR是影响鼻腔NK／T细胞淋巴瘤放疗总生存的有利预后因素。     

Treatment outcome of radiotherapy alone versus radiochemotherapy in early stage nasal natural killer/T-cell lymphoma

This study aims to investigate the prognostic factors and long-term treatment outcome in patients with early stage nasal natural killer (NK)/T-cell lymphoma. Sixty-four patients were recruited in this study, whose clinical and laboratory data were collected from hospital records. Early stage (stage IE: 51, stage IIE: 13) nasal NK/T-cell lymphoma (NNTCL) was established according to Ann Arbor staging classification. Among these patients, 23 received radiotherapy (RT) alone, the remaining 41 cases were treated with radiochemotherapy (RCT) comprised of 1–6 cycles of anthracycline-based chemotherapeutic regimens. Results show that the median overall survival (OS) time was 41 months. The 5-year OS and progression-free survival rates were 59.2 and 52.3%, respectively. The 5-year OS rate for patients who received RT alone was 57.9%, whereas that for patients who received RCT was 61.5% (P = 0.47). There is no significant difference between two treatment modalities. Multivariate analysis showed that Eastern Cooperative Oncology Group performance status (PS) score ≥ 2, local tumor invasion out of nasal cavity, and lower complete remission (CR) rates in the initial treatment were significant unfavorable independent prognostic factors. Taken together, our study suggests that RCT did not improve the survival rate of patients with early stage NNTCL. PS score before treatment, local tumor invasion out of nasal cavity, and CR rate of the primary treatment may be independent prognostic factors among the subtype lymphoma entity.     

Extranodal natural killer/T-cell lymphoma, nasal type: the significance of radiotherapeutic parameters

BACKGROUND: The objective of this study was to investigate the correlation between local recurrence and radiotherapeutic parameters, including dose and RT radiotherapy (RT) field. METHODS: The current study included 35 patients who were diagnosed with immunohistochemically confirmed nasal natural killer (NK)/T-cell lymphoma between 1976 and 2004. There were 21 males and 14 females, and they ranged in age from 18 years to 76 years (median, 51 yrs). The primary tumor originated in the nasal cavity in 28 patients, and 32 patients had Stage I disease. Seventeen patients received treatment solely with RT, and the remaining 18 patients received a combination of chemotherapy and RT. The median tumor dose was 50 grays (Gy) (range, 22-60 Gy). Twenty-seven patients received RT to include all macroscopic lesions, all paranasal sinuses, the palate, and the nasopharynx. Eight patients received RT to all macroscopic lesions with generous margins. RESULTS: A complete remission (CR) or a CR/unconfirmed was achieved in 28 patients (80%). The 5-year overall survival (OAS) rate, disease-free survival (DFS) rate, and local control probability (LCP) were 47.3%, 42.9%, and 65.2%, respectively. Patients who received RT only to macroscopic lesions fared less well in terms of LCP (LCP 5 years, 71.9% vs. 41.7%; P=0.007). The difference in RT field also affected both the OAS rate and the DFS rate. Patients who received RT doses>or=50 Gy tended to achieve favorable local control. CONCLUSIONS: In the management of nasal NK/T-cell lymphoma, the RT field affected treatment outcomes. RT doses>or=50 Gy resulted in favorable local control.