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。     

Peripheral T-cell lymphomas: clinical features and prognostic factors of 92 cases defined by the revised European American lymphoma classification

The purpose of this study was to better define the clinical features and natural history of peripheral T-cell lymphomas (PTCL) entities included in the Revised European American lymphoma (REAL) classification. Cases of PTCL were retrieved from the records of the Department of Pathology and classified according to the REAL classification. In addition, cases of anaplastic large cell lymphoma (ALCL) were divided into classical, small cell, and primary cutaneous subtypes, and immunostaining for the anaplastic large-cell kinase (ALK) protein was performed on all cases of ALCL. Clinical features, response to therapy and survival were abstracted. Ninety-two cases of PTCL with adequate clinical information were retrieved. There were 40 cases of ALCL (30 classical, 7 small cell variant, 3 primary cutaneous), 28 PTCL, unspecified, 13 angioimmunoblastic T-cell lymphoma and 11 with other entities. The patients had a median age of 48 years with a range of 6-84 and had an estimated overall survival (OS) of 49% and progression-free survival (PFS) of 22% at 5 years. The International Prognostic Index (IPI) was a significant prognostic factor for both progression-free and OS. Histology was a significant predictor of PFS with anaplastic large cell having the best prognosis. ALK expression was not associated with an improved progression-free or overall-survival in patients with systemic T-cell ALCL. In conclusion, the REAL classification describes distinct PTCL entities. The IPI is the most important predictor of progression-free and OS in patients with PTCL. ALK expression may not provide prognostic information for systemic ALCL.     

Comparison of four prognostic scores in peripheral T-cell lymphoma

BackgroundTo compare the usefulness of four prognostic scores in patients with peripheral T-cell lymphoma (PTCL) from a single institution.Patients and methodsOne hundred twenty-one patients (77 male/36 female, median age 53 years) with PTCL [anaplastic large-cell lymphoma (ALCL) 21, PTCL not otherwise specified 56 and other 44)]. Complete response (CR) rate and 5-year overall survival (OS) were 41% and 31%, respectively. International Prognostic Index (IPI), Prognostic Index for T-cell lymphoma (PIT), International peripheral T-cell lymphoma Project score (IPTCLP) and modified Prognostic Index for T-cell lymphoma (mPIT) were calculated as in the original references. mPIT was only assembled to 41 patients in whom Ki-67 immunostaining was available. ALCL patients were analyzed separately.ResultsConcordance among IPI, PIT and IPTCLP was 52% for low-risk group, 27% for low/intermediate-risk group, 20% for high/intermediate-risk group and 14% for high-risk group. IPI, PIT and IPTCLP predicted CR, with IPI being the best score in logistic regression. Neither Ki-67 immunostaining nor mPIT predicted CR. Five-year OS (low-risk versus intermediate- or high-risk categories) according to IPI, PIT, IPTCLP and mPIT were 52% versus 45%, 75% versus 49%, 58% versus 20% and 39% versus 0%, respectively. IPTCLP was the best score for OS in multivariate analysis.ConclusionAll the scores demonstrated their usefulness to assess the outcome of patients with PTCL, with IPTCLP being the most significant to predict OS.     

How to predict the outcome in mature T and NK cell lymphoma by currently used prognostic models?

To select an appropriate prognostic model in the treatment of mature T- and natural killer (NK) -cell lymphoma (peripheral T-cell lymphoma (PTCL) and NK-/T-cell lymphoma (NKTCL)) is crucial. This study investigated the usefulness of Ann Arbor staging classification International prognostic index (IPI), prognostic index for T-cell lymphoma (PIT) and International peripheral T-cell lymphoma Project score (IPTCLP). Between 2000 and 2009, 176 patients (122 males) with PTCL and NKTCL were diagnosed and treated from a single institute in Taiwan. The correlation between complete response (CR) rate, 3-year overall survival (OS), early mortality rate and four prognostic models was analyzed. Thirty-one patients received hematopoietic stem cell transplantation (HSCT) and were analyzed separately. Three-year OS rate was 34.7%, and anaplastic large-cell lymphoma harbored better outcome than others. IPI score had the lowest Akaike information criterion value (1081.197) and was the best score in predicting OS and early mortality (P=0.009). Ann Arbor stage classification can predict CR rate more precisely (P=0.006). OS was significantly better in patients who received HSCT, even in patients with unfavorable features compared with chemotherapy alone. All prognostic models were useful to evaluate the outcome of patients with PTCL and NKTCL but IPI score did best in predicting OS in PTCL and PIT score in NKTCL. This study also supported the role of HSCT in patients with high-risk or refractory PTCL or NKTCL.     

Clinico-pathologic features and outcome of Japanese patients with peripheral T-cell lymphomas

We studied the clinico-pathologic features and treatment outcome of patients with peripheral T-cell lymphoma (PTCL). This study included 215 patients with T/natural killer (NK)-cell lymphoma, including 59 with PTCL-unspecified (PTCL-U), 42 with angioimmunoblastic T-cell lymphoma (AILT) and 20 with anaplastic large-cell lymphoma (ALCL). Most of the analyses were performed on patients with AILD, ALCL and PTCL-U. The patients with AILT and PTCL-U tended to be older than those with ALCL. Stage III/IV disease was seen in 90.5% of the AILT cases, 55% of the ALCL cases and 67.8% of the PTCL-U cases. In addition, 61.9% of the AILT cases had an international prognostic index (IPI) of H-I or H risk. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 72.2 and 76.1% among the ALCL cases, 40.7 and 42.2% among the PTCL-U cases and 31.2 and 49.3% among the AILT cases, respectively. Among the patients with PTCL-U, the 5-year PFS and OS rates in group low (L), low-intermediate (L-I), high-intermediate (H-I) or high (H) risk group of IPI were: 47.6 and 56.1%, 55.6 and 53.8%, 42.4 and 40.1% and 9.1 and 9.1%, respectively. The 5-year PFS and OS rates in group 1, 2, 3 or 4 by prognostic index of PTCL-U (PIT) were: 88.9 and 85.7%, 57.1 and 54.9%, 33.5 and 28.8% or 13.3 and 13.3%, respectively. The 5-year PFS and OS rates among patients who received CHOP therapy, CyclOBEAP [cyclophosphamide (CPA), vincristine (VCR), bleomycine, etoposide, doxorubicin (DXR), prednisone (PDN)] therapy or autologous stem cell transplantation were: 22 and 25.7%, 59 and 61.7% or 33.3 and 60%, respectively. Multivariate analysis revealed that the PIT score was associated with OS and PFS. These results indicate that the presence of bone marrow (BM) involvement is an independent prognostic factor which may predict both OS and PFS. PTCL-U is a heterogeneous disease with regard to histological type and pathological state. Because PTCL-U is generally not responsive to CHOP therapy, new treatment strategies need to be developed.     

Peripheral T-cell lymphomas in a large US multicenter cohort: prognostication in the modern era including impact of frontline therapy

BackgroundOptimal frontline therapy for peripheral T-cell lymphoma (PTCL) in the modern era remains unclear.Patients and methodsWe examined patient characteristics, treatment, and outcomes among 341 newly diagnosed PTCL patients from 2000 to 2011. Outcome was compared with a matched cohort of diffuse large B-cell lymphoma (DLBCL) patients, and prognostic factors were assessed using univariate and multivariate analyses.ResultsPTCL subtypes included PTCL, not otherwise specified (PTCL-NOS) (31%), anaplastic large T-cell lymphoma (ALCL) (26%), angioimmunoblastic T-cell lymphoma (23%), NK/T-cell lymphoma (7%), acute T-cell leukemia/lymphoma (6%), and other (7%). Median age was 62 years (range 18-95 years), and 74% had stage III-IV disease. Twenty-three (7%) patients received only palliative care whereas 318 received chemotherapy: CHOP-like regimens (70%), hyperCVAD/MA (6%), or other (18%). Thirty-three patients (10%) underwent stem-cell transplantation (SCT) in first remission. The overall response rate was 73% (61% complete); 24% had primary refractory disease. With 39-month median follow-up, 3-year progression-free survival (PFS) and overall survival (OS) were 32% and 52%. PFS and OS for PTCL patients were significantly inferior to matched patients with DLBCL. On multivariate analysis, stage I–II disease was the only significant pretreatment prognostic factor [PFS: hazard ratio (HR) 0.54, 95% confidence interval (CI) 0.34–0.85, P = 0.007; OS: HR 0.42, 95% CI 0.22–0.78, P = 0.006]. ALK positivity in ALCL was prognostic on univariate analysis, but lost significance on multivariate analysis. The most dominant prognostic factor was response to initial therapy (complete response versus other), including adjustment for stage and SCT [PFS: HR 0.19, 95% CI 0.14–0.28, P < 0.0001; OS: HR 0.26, 95% CI 0.17–0.40, P < 0.0001]. No overall survival difference was observed based on choice of upfront regimen or SCT in first remission.ConclusionsThis analysis identifies early-stage disease and initial treatment response as dominant prognostic factors in PTCL. No clear benefit was observed for patients undergoing consolidative SCT. Novel therapeutic approaches for PTCL are critically needed.     

Histology impacts the outcome of peripheral T-cell lymphomas after high dose chemotherapy and stem cell transplant

The role of high dose chemotherapy (HDC) and stem cell transplant (SCT) in peripheral T-cell lymphoma (PTCL) was studied in 28 patients, from 1988 to 2002. The aim was to determine if subsets recognized by the REAL/WHO classification have different prognoses. Outcome was compared to 86 patients with diffuse large B-cell lymphoma (DLBCL) transplanted during 1986-2000. The 3-year overall survival (OS) and event free survival (EFS) were 69% and 50%. Patients with anaplastic large cell lymphoma (ALCL) had a better 3-year OS compared to those with non-ALCL histology (86% vs. 47%, P=0.0122). Anaplastic lymphoma kinase (ALK)- positive ALCL patients had a superior EFS compared to ALK-negative ALCL (100% vs. 0; P=0.0228). Patients with cutaneous ALCL (ALK-negative) relapsed, but had an indolent course after SCT. Low International Prognostic Index score at relapse predicted for a better 3-year OS (85% vs. 34%, P=0.0238). When compared to DLBCL, patients with ALCL had a superior OS (86% vs. 36%, P=0.0034) and patients with non-ALCL had a comparable OS. ALCL histology confers better survival compared to non-ALCL and DLBCL histologies. ALK-positive ALCL is associated with the best EFS after relapse with HDC and SCT. The timing of SCT for non-ALCL histology remains to be determined.     

Stem cell transplantation for peripheral T-cell lymphomas

Peripheral T-cell lymphomas (PTCL) consist of many subtypes with variable clinical presentation. Long-term prognosis of most subtypes is unfavorable and novel therapeutic approaches are needed. This review attempts to summarize what is known on the feasibility and efficacy of high-dose therapy supported by stem cell transplantation (SCT) in PTCL. In patients with relapsed or refractory PTCL, the outcome of autologous SCT (ASCT) seems to be comparable to that of patients with aggressive B-cell lymphomas. Although excellent treatment results have been encountered with ASCT in patients with anaplastic large cell lymphoma (ALCL), the superiority of this approach over chemotherapy alone needs confirmation in randomized studies. In less favorable subtypes (e.g. alk-negative ALCL, PTCL not otherwise specified, enteropathy-associated T-cell lymphoma, and angioimmunoblastic T-cell lymphoma) high-dose consolidation of the first remission should be studied in prospective trials. Minimal experience is currently available on allogeneic SCT in patients with PTCL. Given the high relapse rate after ASCT in high-risk patients and potential for graft-vs.-lymphoma effect, also this approach should be studied. Due to rarity of PTCL, international collaboration is mandatory in order to study the various aspects of SCT in this patient population.     

外周T细胞淋巴瘤血清β2-微球蛋白水平与国际预后指数的关系及预后分析

 【摘要】　目的　探讨血清β2-微球蛋白（β2-MG）水平与国际预后指数（IPI）的关系,及其对外周T细胞淋巴瘤（PTCL）预后判断的应用价值,为个体化治疗提供依据。方法　回顾性分析采用CHOP方案治疗的81例PTCL的临床资料,对β2- MG水平、IPI评分系统与疗效及远期生存的关系进行统计学分析。结果　全组81例患者,均接受CHOP方案联合化疗,总有效（RR）率为82.7 %,完全缓解（CR）率53.1 %。IPI评分低危组、低中危组、中高危组及高危组的RR率分别为95.7 %、87.5 %、53.8 %和20.0 %,CR率分别为74.5 %、37.5 %、15.4 %和0,各组间差异均有统计学意义（均P＜0.05）。中位随访时间30个月（2～98个月）,中位生存期（MST）31.2个月,总的1、3、5年生存（OS）率分别为83.5 %、41.8 %和34.7 %。低危和低中危组MST目前尚未达到,中高危和高危组MST分别为16、7个月,5年OS率分别为57.3 %、55.9 %、0和0,差异有统计学意义（P＜0.05）。将IPI评分0～1分和2分合并为低危组,3分、4～5分合并为高危组,进行生存比较,前者MST尚未达到,后者为11个月,5年OS率分别为54.8 %和0（P＜0.05）。高危组血清β2- MG水平均较低危组明显升高,高危组患者较低危组患者血清β2-MG异常者比例也明显升高（均P＜0.05）。多因素分析结果显示血清β2- MG水平、IPI评分与PTCL的预后密切相关（P＜0.05）。结论　血清β2- MG水平可以和IPI评分系统一起用于外周T细胞淋巴瘤患者的预后判断。     

High-dose chemotherapy and autologous stem cell transplantation in peripheral T-cell lymphoma: the GEL-TAMO experience.

BACKGROUND: T-cell immunophenotype constitutes an unfavorable prognostic factor in aggressive non-Hodgkin's lymphomas. High-dose chemotherapy with autologous stem-cell rescue (HDC/ASCT) is the best salvage therapy for patients with aggressive B-cell lymphomas. However, results with this therapy in peripheral T-cell lymphoma (PTCL) are not well defined. PATIENTS AND METHODS: From January 1990 to December 1999, 115 patients with PTCL underwent HDC/ASCT inside the Grupo Espa?ol de Linfomas/Trasplante Autólogo de Médula Osea (GEL-TAMO) registry. At diagnosis the median age was 41 years and 60% of patients presented with two or three risk factors from the adjusted International Prognostic Index (a-IPI). Thirty-two per cent of patients were transplanted in first complete response (CR), 62% in chemosensitive disease and 5% in refractory disease. RESULTS: Eighty-six per cent of the patients attained a CR and 5% a partial response (PR). With a median follow-up of 37 months (range 1-133), overall survival (OS), time-to-treatment failure (TTF) and disease-free survival (DFS) at 5 years was 56%, 51% and 60%, respectively; for the 37 patients transplanted in first CR, OS and DFS at 5 years were 80% and 79%, respectively. Lactase dehydrogenase (LDH), a-IPI and disease status pre-transplant were associated with outcome. CONCLUSIONS: More than half of patients with chemosensitive disease who were transplanted are expected to be alive at 5 years. We confirm the utility of the pre-transplant IPI system in predicting outcome. Salvage treatment results with HDC/ASCT in PTCL are similar to those found in corresponding aggressive B-cell lymphomas.     

原发结内外周T细胞淋巴瘤19例临床特征及预后分析

 目的　分析原发结内外周T细胞淋巴瘤（PTCL）的临床特点、治疗和预后。方法　回顾性分析19例原发结内PTCL患者的临床资料、治疗反应以及预后因素。结果　19例患者中位发病年龄54岁,男女比例2.17∶1,其中94.7 %（18／19）为Ⅲ～Ⅳ期,84.2 %（16／19）有B症状,84.2 %（16／19）有结外器官受累,57.9 %（11／19）有骨髓浸润。化疗完全缓解（CR）率36.8 %（7／19）,2年总生存（OS）率47.4 %,2年无进展生存（PFS）率25 %。预后分析显示,结外侵犯数量（EN）≥2个、美国东部肿瘤协作组（ECOG）体能状态评分≥2分、国际预后指数（IPI）评分＞2分以及β2-微球蛋白（β2-MG）升高为不良预后因素。结论　原发结内PTCL是一类高度侵袭的异质性T细胞淋巴瘤,化疗效果差,多项因素提示不良预后。     

The results of consolidation with autologous stem-cell transplantation in patients with peripheral T-cell lymphoma (PTCL) in first complete remission: the Spanish Lymphoma and Autologous Transplantation Group experience.

BACKGROUND: Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of aggressive lymphomas associated with poor prognosis with standard chemotherapy. Consolidation with autologous stem-cell transplantation (ASCT) may improve survival. We present 74 patients transplanted in first complete response (CR) from the Spanish Lymphoma and Autologous Transplantation Group cooperative group. PATIENTS AND METHODS: Median age was 46 years. Eighty-eight percent presented advanced (III-IV) Ann Arbor stage; 53% had B symptoms; 52% had high lactate dehydrogenase; 65% had two or three risk factors of the adjusted-International Prognostic Index; 58% presented a high Tumor score and in 14% more than two adverse factors of the Prognostic Index for peripheral T-cell lymphoma (PIT) were observed. RESULTS: With a median follow-up of 67 months from diagnosis, the 5-year overall survival (OS) was 68% and progression-free survival (PFS) reached 63%. The multivariate analysis showed that the only factor associated with a shorter OS and PFS was the presence of more than two risk factors from the PIT risk system. CONCLUSIONS: In a retrospective study with a prolonged follow-up, consolidation with ASCT in CR patients who had presented unfavorable prognostic factors at diagnosis substantially increased the OS and PFS when compared with conventional chemotherapy. The PIT risk system identified 14% of patients without benefit from ASCT consolidation. Thus, other innovative therapies are still necessary in certain cases.     

EPOCH方案治疗外周 T细胞非霍奇金淋巴瘤的临床报告

背景和目的：外周T细胞淋巴瘤(peripheral T-cell lymphoma,PTCL)与B细胞非霍奇金淋巴瘤(non-Hodgkin's lymphoma,NHL)相比,往往表现为化疗敏感性差、容易复发、预后不良,目前尚无标准化疗方案。CHOP(CTX、ADM、VCR、prednisone)方案治疗PTCL疗效不理想,本试验旨在探索持续静脉灌注EPOCH(VP-16、EPI／ADM、VCR、CTX、prednisone)方案治疗PTCL的疗效和不良反应。方法：在2001年11月～2004年4月期间,共收治PTCL21例,其中7例外周T细胞淋巴瘤-非特指型(PTCL-U),7例鼻型NK／T细胞淋巴瘤(NK／TCL),5例间变大细胞淋巴瘤(ALCL),1例蕈样肉芽肿／Sezary综合征(MF／SS),1例皮下脂膜炎样T细胞淋巴瘤(SPTCL);其中14例初治,7例复治。所有患者接受EPOCH化疗1～7个疗程(中位疗程数3个)。结果：21例PTCL患者中20例可评价疗效,总的客观有效率(RR)85％(17／20),完全缓解率(CR率)50％(10／20)。其中NK／T有效率71．4％(5／7),CR率57．1％(4／7);PTCL-U有效率100．0％(6／6),CR率为50．0％(3／6);ALCL有效率80．0％(4／5),CR率为40．0％(2／5)。初治者有效率84．6％(11／13),CR率为61．5％(8／13);复治者有效率85．5％(6／7),CR率为28．5％(2／7)。21例患者共实施70个疗程化疗,主要不良反应为骨髓抑制,其中Ⅲ～Ⅳ度粒细胞减少为34．3％,40个疗程需G-CSF支持,Ⅲ～Ⅳ度血小板减少为14．3％,Ⅲ～Ⅳ度贫血为7．1％。其他不良反应少见,无治疗相关死亡。结论：采用EPOCH方案治疗PTCL疗效好,容易耐受,值得进一步研究。     

非霍奇金淋巴瘤1012例临床病理分析

目的:了解我院近10年来非霍奇金淋巴瘤(NHL)的发病特点,分析影响NHL预后的相关因素.方法:回顾性分析了近10年来我院收治的1012例NHL患者的临床病理特点,对影响新疆地区NHL生存率及预后的临床病理因素进行分析.结果:1012例NHL中以40～60岁汉族男性发病多见,最主要病理类型依次为弥漫性大B细胞淋巴瘤(DLBCL)346例(34.1%),外周T细胞淋巴瘤(PTCL)185例(18.3%),滤泡淋巴瘤(FL)97例(9.6%),黏膜相关淋巴组织淋巴瘤(MALT)94例(9.3%),NK/T细胞淋巴瘤62例(6.1%),T-淋巴母细胞淋巴瘤(T-LBL)47例(4.6%).结性起病的淋巴瘤619例(61.2%),结外起病的淋巴瘤393例(38.8%).本组维吾尔族女性FL淋巴瘤患者人数比例较汉族女性患者高(P=0.002),汉族男性PTCL患者人数比例高于维吾尔族男性患者(P=0.015).5年总生存率为45.8%.单因素分析显示临床分期,行为状态评分(PS),B症状,年龄,肿块大小,血清乳酸脱氢酶(LDH),结外器官受侵数目及IPI是NHL的预后因素(P＜0.05).多因素分析提示T细胞来源,Ⅲ～Ⅳ临床分期,IPI评分3～5分及LDH增高是NHL独立的预后不良因素(P＜0.05).结论:新疆地区NHL发病以中年多见,结性起病者多于结外起病,B细胞淋巴瘤多于T细胞淋巴瘤.免疫分型、临床分期、IPI、血清LDH水平与NHL预后相关.     

外周T细胞淋巴瘤合并自身免疫性溶血性贫血的临床特点分析

背景与目的:与自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)相关的淋巴瘤病理学类型多见于惰性B细胞淋巴瘤,而很少见于弥漫性大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)及外周T细胞淋巴瘤(peripheral T-cell lympho...     

T-cell non-Hodgkin's lymphoma in adults: clinicopathological characteristics,response to treatment and prognostic factors

T-cell NHL represent 10-15% of all malignant lymphomas making systematic prospective clinical trials difficult. Therefore, the prognostic significance of the T-cell phenotype has been a matter of controversy in recent years. In a retrospective analysis of 681 patients (pts) with NHL accrued from 1992 to 1997 at a single institution, 66 patients with T-cell NHL were identified. According to the REAL classification, histologies were as follows: 28 peripheral T-cell lymphomas (PTCL), 19 large cell anaplastic lymphoma (LCAL), 12 precursor lymphoblastic lymphoma (Lb), and seven AILD. Multiagent anthracycline containing regimens were used as initial therapy in 91% of cases. T-cell NHL represent 9.8% of all NHL patients at our institution accrued over a 6-year period. Overall response rate was 76%, 21% had progressive disease and 3% died during first line treatment. Mean overall survival (OS) was 8.22 +/- 0.94 years. There was a significant difference in OS between the four different histological subgroups (log rank P=0.0288). LCAL: mean OS 11.05 +/- 1.55 years (95% CI 8.00-14.09); LB: mean OS 7.09 +/- 1.40 years (95% CI 4.33-9.84); PTCL: mean 6.62 +/- 1.17 years (95% CI 4.33-8.90); AILD: 1.54 +/- 0.44 years (95% CI 0.67-2.40). OS was also significantly different for patients classified according to the International Prognostic Index (IPI)-score (log rank P = 0.002). Mean OS for patients with low risk, intermediate low risk, intermediate high risk and high risk was 10.46 +/- 1.02, 6.46 +/- 1.79, 4.50 +/- 1.20 and 1.15 +/- 0.46 years, respectively. Univariate analysis (log-rank test) for prognostic factors also revealed elevated LDH, B-symptoms and extranodal involvement as significant factors for OS. The presence of bulky disease (>7.5 cm), advanced stage III/IV and bone marrow involvement did not influence OS. In conclusion, it is evident that histological subtype and IPI-score have a strong prognostic impact on OS in pts with T-cell NHL. Thus, the distribution of risk factors in patients with T-cell NHL may be more important for OS than T-cell histology per se.     

Long-term follow-up of patients with peripheral T-cell lymphomas treated up-front with high-dose chemotherapy followed by autologous stem cell transplantation.

We report the results of two prospective phase II studies investigating the role of high-dose sequential chemotherapy, followed by autologous stem cell transplantation (ASCT) in 62 patients with advanced stage peripheral T-cell lymphomas (PTCLs) at diagnosis. Conditioning regimen consisted of mitoxantrone (60 mg/m2) and melphalan (180 mg/m2) or carmustine, etoposide, Ara-C and melphalan followed by peripheral blood stem cell autografting. In an intent-to-treat analysis, 46 out of 62 patients (74%) completed the whole programme, whereas 16 patients did not undergo ASCT, mainly because of disease progression. At a median follow-up of 76 months, the estimated 12-year overall (OS), disease-free and event-free survival (EFS) were 34, 55 and 30%, respectively. OS and EFS were significantly better in patients with anaplastic lymphoma-kinase (ALK)-positive anaplastic large-cell lymphoma (ALCL), as compared with the remaining PTCL. Multivariate analysis showed that patients attaining complete remission (CR) before ASCT had a statistically significant benefit in terms of OS and EFS (P<0.0001). Overall treatment-related mortality rate was 4.8%. In conclusion, our findings indicate (1) up-front high-dose therapy and ASCT are feasible, but could induce a high rate of long-term CR only in patients with ALK-positive ALCL and (2) the achievement of CR before autografting is a strong predictor of better survival.     

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.     

Treatment of Peripheral T-Cell Lymphoma in Community Settings

BackgroundPeripheral T-cell lymphomas (PTCLs) represent a rare and heterogeneous group of malignancies that do not have consensus treatment recommendations. Strategies extrapolated from B-cell lymphoma have met with limited efficacy, although T-cell–specific salvage therapies have been recently developed.MethodsTo determine treatment patterns and associated outcomes in PTCL not otherwise specified (PTCL-NOS), anaplastic large T-cell lymphoma (ALCL), and angioimmunoblastic T-cell lymphoma (AITL), a retrospective analysis was undertaken at a large US community oncology network among patients treated between January 2010 and April 2015.ResultsAmong 93 patients (44 PTCL-NOS, 30 ALCL, 19 AITL), 23 unique treatments were used in 66 first-line patients and 12 unique second-line treatments were used in 24 relapsed/refractory patients. First-line CHOP and CHOP-like regimens were used in 74% of patients, providing 4-year overall survival (OS) outcomes of 34% (95% confidence interval [CI], 14%-83%) in patients without transplant consolidation (82% in ALCL, 37% in PTCL-NOS, and 0% in AITL). Upfront stem cell transplantation trended toward improved 4-year progression-free survival 77% (95% CI, 54%-100%) versus 34% (95% CI, 14%-80%); (P = .08; hazard ratio [HR] 0.29) with 4-year OS 77% (95% CI, 54%-100%) versus 34% (P = .22; HR 0.41). Brentuximab was the most common second-line therapy, with multiple additional regimens used in sequence (up to 5 salvage regimens) in many.ConclusionsThe significant variability in treatments used for PTCL emphasizes the lack of consensus therapy in this rarer lymphoma and calls for additional organized prospective and registry studies to evaluate comparative effectiveness.     

国际预后指数在外周T细胞淋巴瘤-非特异型预后判断中的意义

 目的　评价国际预后指数（IPI）在外周T细胞淋巴瘤-非特异型（PTCL-NOS）预后判断中的价值。方法　分析2005年5月至2008年5月间75例经免疫组织化学重新确诊的PTCL-NOS患者临床资料，按IPI进行低危（0～1分）、低中危（2分）、中高危（3分）、高危（4～5分）分组，分析不同的危险级别对治疗的反应及预后的差异。结果　75例中，根据IPI分成的4组（低危、低中危、中高危、高危组）分别为10、14、28、23例；占13.3%、18.7%、37.3%、37.0%。4组之间的首程治疗的完全缓解率（χ2＝16.677，P＝0.001）、总的生存率（OS）（P＝0.0000）差异均有统计学意义。4组的中位生存时间（MST）分别为：36＋、29.00、17.00、10.00个月。4组的1年OS分别为：100.00 %、89.05 %、64.24 %、15.73 %。2年的OS分别为75.00 %、53.01 %、34.42 %、2.00 %。多因素生存分析显示，首程化疗的完全缓解率（P＝0.002）和IPI评分（P＝0.049）可以作为PTCL-NOS的独立预后因素，而IPI中的单一指标尚不能作为独立的预后因素。结论　IPI能够在一定程度上预测PTCL-NOS对治疗的反应性和预后。