129例原发鼻腔非霍奇金淋巴瘤的预后分析

背景与目的：原发鼻腔非霍奇金淋巴瘤（non-Hodgkin’s lymphoma,NHL）预后较差．远处转移和局部复发率均较高．目前本病尚无标准治疗原则。本研究主要探讨影响原发鼻腔NHL的预后因素。方法：1996年1月至2002年12月共收治129例经病理证实的原发鼻腔NHL。116例经病理形态学诊断为鼻腔NK／T细胞淋巴瘤。根据Ann Arbor分期,ⅠE期102例,ⅡE期22例,ⅣE期5例。124例ⅠE／ⅡE期患者单纯放疗22例,单纯化疗7例,45例放疗后化疗,50例化疗后放疗。5例ⅣE期以化疗为主。结果：全组5年总生存率（overall survival,OS）和无病生存率（disease-freely survival,DFS）分别是68．0％和55．8％。ⅠE期和ⅡE期患者的5年OS分别是71．7％和70．6％（P=0．77）,DFS分别是60．9％和47．0％（P=0．09）。首程治疗后完全缓解（complete response,CR）的患者5年OS为83．1％,而未达CR患者的5年OS为18．0％（P〈0．01）,相应5年DFS分别为68．0％和15．5％（P〈0．01）。国际预后指数（international prognostic index,IPl）评分为0,1和≥2的5年OS分别为81．1％、60．1％和14．3％（P〈0．01）,DFS分别为68．8％、44．6％和22．5％（P〈0．01）。38例患者治疗中进展或疗后复发,远处结外器官复发占78．9％,是治疗失败主要原因。单因素分析显示,首程治疗后的CR率、一般状态评分（performance status,PS）、IPI和修订后IPI与预后相关。多因素分析显示仅治疗后的CR率是独立预后因素。结论：治疗后的CR率是影响原发鼻腔NHL的重要预后因素,原因以远处病灶治疗失败为主。     

Diffuse large B-cell lymphoma: clinical and biological characterization and outcome according to the nodal or extranodal primary origin.

PURPOSE: To study the main clinicobiologic features, response, and outcome of patients with diffuse large B-cell lymphoma (DLBCL) according to the primary site, lymph node, or different extranodal organs of the disease. PATIENTS AND METHODS: We included 382 patients consecutively diagnosed with DLBCL in a single institution during a 13-year period. Morphology, immunophenotyping, proliferation index, differentiation profile, bcl-2/JH rearrangement, and clinical characteristics were analyzed according to the primary site of the lymphoma. RESULTS: Sites of the disease were: lymph node, 222 cases (58%); Waldeyer's ring (WR), 42 (11%); and extranodal sites, 118 (31%), including GI tract in 45 cases. Primary extranodal cases, particularly GI, showed a bcl-6 expression more frequently than nodal cases. Patients with primary WR or GI lymphomas presented with early-stage disease, no marrow infiltration, normal serum lactate dehydrogenase, and low- to low/intermediate-risk international prognostic index (IPI) more frequently than the remainder. Complete response (CR) rate was 63%, with WR and GI lymphomas having a higher CR rate (85% and 80%, respectively) than the other groups. In the whole series, 5-year overall survival (OS) was 52%. Patients with WR or GI lymphomas showed better OS (5-year OS: 77% and 68%, respectively) than patients with nodal or other extranodal sites. In the multivariate analysis, IPI, bulky disease, and beta2-microglobulin were the main variables to predict OS; no nodal or extranodal site maintained their prognostic value. CONCLUSION: In the present series, the primary site of disease was associated with particular clinicopathologic features and outcome, though the latter largely depended on other factors.     

Helicobacter pylori and carcinogenesis of gastric B-cell lymphomas

Primary non-Hodgkin's lymphomas of the stomach are associated with Helicobacter pylori infection. We analyzed gastric lymphoma onset data with respect to prior H. pylori infections based on the multistage theory of carcinogenesis. This theory provides a link between epidemiological data and biological processes. The study involved 133 patients, aged 29-75 years, diagnosed with marginal zone B-cell lymphoma (MZBL) and diffuse large cell B-cell lymphoma (DLBL). A 2-parametric Weibull model was applied to MZBL and DLBL onset data. Median age of diagnosis of MZBL (DLBL) was 59 years (55 years) in males and 65.5 years (64 years) in females. Infection with H. pylori was found in 81.3% (59.5%) of the patients diagnosed with MZBL (DLBL). Lymphoma latency data were fitted to Weibull distributions with a shape parameter of 5.7 for MZBL cases and 4.2 for DLBL. The shape parameter that indicates the number of steps in carcinogenesis was approximately independent of the status of infection with H. pylori in DLBL in contrast to MZBL. It was shown that gastric lymphoma onset data can be described by Weibull distribution functions. The findings support the hypothesis that MZBL and DLBL have different lines of development. There is indication of stronger antigen dependency in the carcinogenesis of MZBL in comparison to DLBL.     

High-dose chemotherapy with autologous hematopoietic stem cell transplantation for non-Hodgkin's lymphoma in complete response as consolidation therapy, second report

High-dose chemotherapy followed by autologous peripheral blood transplantation (HD-APBSCT) is a therapeutic option for patients with non-Hodgkin's lymphoma (NHL) after complete remission (CR) as consolidation therapy. In this report we describe a retrospective study of such treatment. A total of 38 patients with NHL were treated between November 19 9 1 and March 2005. At five years,the rate of disease-free survival (DFS) and overall survival (OS) was 64.3% and 66.5%, respectively. Patients who underwent transplantation in first CR had a 5-year probability of disease-free survival of 71.6% compared with 35.7% for those who were in second CR at the time of transplantation (p=0.10). In a monovariate analysis, second CR status at the time of transplantation was a relatively adverse predictor of DFS. None of those factors containing surface markers were significantly associated with clinical variables such as the CR status at the time of transplantation. Thirty high intermediate risk and high risk patients with aggressive B-cell lymphoma had a better outcome than patients treated with standard chemotherapy. In this study, 8 patients with T-cell lymphoma had a 3-year DFS and OS of 87.5% and 87.5%, respectively. HDT-APBSCT is a candidate for consolidation therapy for high-intermediate risk and high risk patients with aggressive B-cell and T-cell lymphoma.     

Different age limits for elderly patients with indolent and aggressive non-hodgkin lymphoma and the role of relative survival with increasing age

BACKGROUND: There is no consistent definition at what age patients with non-Hodgkin lymphoma (NHL) are considered "elderly." This might hamper well balanced decisions with respect to treatment. METHODS: From a population-based NHL registry the age groups younger than 60 years, 60-64 years, 65-69 years, 70-74 years, and 75 years and older were analyzed in relation to the revised European-American lymphoma classification and to the age-adjusted International Prognostic Index (IPI). The prognostic value of the variables from the age-adjusted IPI was determined. The relative survival probabilities were calculated. RESULTS: The incidence of diffuse large B-cell lymphoma (DLBL) increased with advancing age, as was the case for small lymphocytic lymphomas. Follicular lymphomas were less frequently encountered with advancing age. With respect to the so-called indolent lymphomas, a decreasing complete remission rate and overall survival rate (5-year) was observed for patients older than 70 years, whereas patients with DLBL fared worse when older than 65 years and 60 years, respectively. The age-adjusted IPI score was discriminative for prognosis. However, even with an IPI score nil, the age group older than 75 years fared significantly worse (P < 0.009), but less so with the relative survival model. The relative survival at 5 years was 60%, 53%, 48%, 35%, and 32% for the 5 respective age groups. CONCLUSIONS: Patients with indolent lymphomas become elderly when they are older than 70 years, but when aggressive lymphoma is concerned this occurs when patients are older than 65 years. For patients with an IPI score nil, age older than 75 years is the dominant prognostic factor. The negative influence of concomitant disease on overall survival, although continuously increasing in older age groups, seems to diminish for patients older than 75 years when compared with the general Dutch population.     

Incidence of gastric B-cell lymphomas: a population-based study in Germany.

BACKGROUND: While the clinical and experimental knowledge concerning gastric lymphomas is increasing, there is a scarcity of epidemiological data. PATIENTS AND METHODS: A population-based sample of patients in Franconia and Saarland in Germany was collected from a clinical trial, hospital archives and a cancer registry. RESULTS: Over a period of 3 years, 94 patients with primary gastric lymphoma were recorded out of a total population of 3.5 million. The standardised incidence rates in Saarland and Franconia were 0.7 and 0.8 cases per 100 000, respectively. Patients were predominantly from higher age groups (mean age 62.1 years) and the incidence in men was slightly more than in women (P <0.03). The distribution of histological subtypes in Franconia was as follows: marginal zone B-cell lymphomas (MZBL), 58%; diffuse large-cell B-cell lymphoma (DLBL), 33%; and mixed forms, 9%. Helicobacter pylori could be detected histologically in 84% of all cases, 95% of MZBL cases and 68% of DLBL cases. CONCLUSIONS: Incidence rates of gastric lymphoma in Germany were similar to that in other European countries, except England, where rates are lower. The subtype-specific differences of H. pylori infection rates could be due to differences in carcinogenesis or to secondary changes during malignant transformation.     

DA-EPOCH-R方案治疗B细胞非霍奇金淋巴瘤效果观察

目的 观察DA-EPOCH-R方案治疗B细胞非霍奇金淋巴瘤(NHL)的效果及安全性.方法 采用DA-EPOCH-R方案治疗43例B细胞NHL患者,观察近期疗效、不良反应,进行随访,分析生存情况.结果 43例NHL患者共接受203个疗程化疗,中位化疗6个(2~8个)疗程.32例(74.4%)化疗2~4个疗程后达完全缓解(CR).年龄≤60岁与>60岁、 Ⅰ~Ⅱ期与Ⅲ~Ⅳ期、 生发中心来源(GCB)型与非GCB型、双表型与非双表型患者CR率差异均无统计学意义(均P>0.05).中位随访40个月(9~62个月),1、3年总生存率分别为97.6%、92.8%.不良反应为血液学不良反应,化疗结束后的随访期内未见严重不良反应,未出现继发性第二肿瘤.结论 DA-EPOCH-R方案治疗B细胞NHL CR率较高,Ⅲ、Ⅳ期患者的疗效与Ⅰ、Ⅱ期相近,老年患者耐受性良好.     

Long-term outcome of mesna,ifosfamide, mitoxantrone,etoposide (MINE) regimen as a consolidation in patients with aggressive non-Hodgkin lymphoma responding to CHOP

In aggressive non-Hodgkin lymphoma (NHL), CHOP (cyclophosphamide, vincristine, doxorubicin, prednisolone) regimen has been standard for decades, and rituximab has increased response rates and survival in CD20 positive patients, recently. The aim of this prospective trial was to evaluate the long-term efficacy and toxicity of MINE as a consolidation treatment in aggressive NHL patients who had achieved CR or unproven CR after six cycles of CHOP in the first line setting. The primary end-point was disease-free-survival (DFS). Thirty-eight patients were enrolled between February 1992 and May 2000. All of the patients received two cycles of MINE (mesna 1.3 g/m², ifosfamide 1.3 g/m², etoposide 65 mg/m² on days 1–3, and mitoxantrone 12 mg/m² on day 1, every 3 weeks) following response to CHOP. Initial bulky disease sites were also applied radiotherapy. Male/female ratio was 1.53(23/15). Median age was 49(30–73). Most of the patients had advanced stage (84.2% for stage >3) and high IPI score (79% for IPI score >2). Sixty percent had diffuse large cell histology. Median follow-up time was 118 months (9–195). Actual mean dose intensity was 88%. There were seven febrile neutropenia episodes. Two patients had grade two neuropathy, one had grade three mucositis and another one had non-neutropenic pneumonia. There was no early toxic death. No serious late toxicity was observed during long-term follow-up. Five- and 10-year DFS rates were both 65.3%. DFS rate in the patients with more than two poor prognostic factors according to IPI score is remarkably high (88%). Five- and 10-year OS was 62.5 and 59%, respectively. MINE regimen seems to be effective as a consolidation regimen, especially, in intermediate/high risk patients and has low early and late toxicities, and it warrants to be evaluated in phase III randomised trials with rituximab in CD20 positive aggressive NHL patients.     

Antiviral treatment in patients with indolent B-cell lymphomas associated with HCV infection: a study of the Fondazione Italiana Linfomi

BackgroundTumor regression after antiviral therapy (AT) is in favor of an etiological role of hepatitis C virus (HCV) in non-Hodgkin's B-cell lymphomas (NHL).Patients and methodsWe carried out a cohort study of 704 consecutive HIV-negative, HCV-positive patients with indolent NHL diagnosed and treated from 1993 to 2009 in 39 centers of the Fondazione Italiana Linfomi; 134 patients were managed with AT for lymphoma control.ResultsFor entire cohort, 5-year overall survival (OS) was 78% [95% confidence interval (CI): 74%–82%] and 5-year progression-free survival (PFS) was 48% (95% CI: 44%–53%). In multivariate analysis, the use of AT during the patients’ life had positive impact on OS. Forty-four of the 100 patients treated with first-line AT achieved a complete remission (CR) and 33 a partial response (PR). HCV-RNA clearance was achieved in 80 patients and was related to lymphoma response. At a median follow-up of 3.6 years, 5-year PFS was 63% (95% CI: 50%–73%). CR + PR rate was 85% with AT as second-line treatment.ConclusionAT produces HCV-RNA clearance and consequent tumor regression in most patients with HCV-related indolent NHL. AT used at any time is associated with improved OS. Consequently, AT can be considered an option for patients with indolent lymphomas who do not need immediate cytoreductive treatment.     

Prognostic significance of the immunophenotype versus the International Prognostic Index in aggressive non-Hodgkin's lymphoma

The International Prognostic Index (IPI) is currently the most widely accepted prognostic factor system for patients with aggressive non-Hodgkin's lymphoma (NHL). However, in constructing the model, the immunophenotype of the disease was not used as an independent variable. The purpose of the present study was to assess and compare the prognostic significance of the immunophenotype (B-cell vs. T-cell) of aggressive NHL with other well-established prognostic determinants, in particular the IPI. Between January 1995 and December 2000, a retrospective analysis was conducted of clinical and pathological data on 181 patients aged = 15 years who had been newly diagnosed with aggressive NHL. All pathology slides were reviewed and defined according to the Revised European-American Lymphoma classification. Forty-one patients (23%) had T-cell lymphoma and 140 patients (77%) had B-cell lymphoma. Diffuse large B-cell lymphoma and unspecified peripheral T-cell lymphoma were the 2 most common entities, comprising 63% and 14% of patients, respectively. Most of the pretreatment characteristics, including IPI risk groups, were not significantly different between B-cell and T-cell lymphomas. The rates of complete remission (71% vs. 54%, P = 0.038) and progressive disease (39% vs. 63%, P = 0.023) significantly favored patients with B-cell lymphoma. With a median follow-up time of 31 months (range, 10-81 months), the 5-year overall survival (49% vs. 27%; P < 0.001) and event-free survival (35% vs. 10%; P < 0.001) were significantly better in B-cell lymphoma. The 5-year disease-free survival was also in favor of the B-cell group (48% vs. 21%; P = 0.086). Patients with T-cell lymphoma yielded inferior survival in all IPI risk groups. Multivariate analysis revealed T-cell lymphoma as the most significant factor associated with short overall survival (relative risk [RR], 3.4; 95% CI, 1.9-5.9) and event-free survival (RR 2.7, 95% CI, 1.7-4.3). When a second multivariate analysis was done using IPI (age, stage, performance status, number of extranodal sites, and serum lactate dehydrogenase) as one independent variable, T-cell phenotype remained the strongest factor affecting the survival of patients (P < 0.001). T-cell lymphoma is an independent prognostic factor, the significance of which is at least comparable to the IPI for patients with aggressive NHL.     

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.     

Ophthalmologic and intraocular non-Hodgkin's lymphoma: a large single centre study of initial characteristics, natural history, and prognostic factors

The aims of this study were to define the initial characteristics, natural history, and prognostic factors of patients with ophthalmologic and intraocular malignant lymphoma. All patients treated at the Institut Curie for lymphoma with ophthalmologic (orbit and/or adnexa) or intraocular involvement were retrospectively reviewed. A pathological review of all cases was performed according to the WHO classification. One hundred and forty-five patients were selected for the study. Pathological review showed 36% MALT type lymphoma, 22% lymphoplasmocytic lymphoma, and 15% diffuse large B-cell lymphoma. Ophthalmologic and ocular sites were intra-orbital in 61 cases (42%) and conjunctival in 51 cases (35%), with bilateral involvement in 10% of cases. Stage IV was found in 32% of cases, with bone marrow involvement in 12%. With a median follow-up of 90 months, the 5-year DFS and OS were 64 and 79% for low-grade NHL, and 43 and 50% for high-grade NHL. On multivariate analysis, age greater than 59 years, elevated LDH level, stage IV, high-grade histological subgroup, and presence of B-symptoms had a negative impact on OS for the overall population. In conclusion, with a median follow-up of 7.5 years, our large cohort of patients represents one of the largest published series on primary ophthalmologic and intraocular malignant lymphoma.     

原发鼻腔非霍奇金淋巴瘤的治疗选择和疗效

目的 分析原发鼻腔非霍奇金淋巴瘤（NHL）放疗和化疗的近期疗效以及治疗方法对预后的影响。方法 129例经病理证实的原发鼻腔NHL患者中,经形态学诊断为鼻腔NK／T细胞淋巴瘤者116例。做免疫组化57例,其中52例为NK／T细胞来源,占91．2％;5例为B细胞来源,占8．8％。根据Ann Arbor分期,ⅠE期102例,ⅡE期22例,ⅣE期5例,ⅠE和ⅡE期患者中,单纯放疗22例,单纯化疗7例,综合治疗95例,ⅣE期以化疗为主。结果 5年总生存率（OS）和无病生存率（DFS）分别为68．0％和55．8％,ⅠE期和ⅡE期患者的5年OS分别为71．7％和70．6％（P=0．77）,DFS分别为60．9％和47．0％（P=0．09）。首程治疗后达CR患者的5年OS为83．1％,而未达CR患者的5年OS为18．0％（P=0．000）,相应5年DFS分别为68．0％和15．5％（P=0．000）。124例ⅠE和ⅡE期患者中,67例患者接受单纯放疗或放疗后化疗,放疗后完全缓解率（CR）为74．7％,其余57例为化疗后放疗或单纯化疗,化疗后CR率仅19．3％（P=0．000）,46例化疗后未达CR的患者中,42例仍局限于局部区域,31例经放疗达到CR,ⅠE和ⅡE期患者中,先放疗组（放疗＋化疗或单纯放疗）、化疗后放疗组的5年OS分别为76．0％和74．4％,DFS分别为65．0％和56．2％（P〉0．05）,ⅠE和ⅡE期单纯化疗7例,3例存活,4例死亡,中位生存时间15个月,1年生存率为26．7％。结论 中国人原发鼻腔NHL主要为NK／T细胞来源,放疗的近期疗效显著优于化疗,化疗加入放疗并未改善生存率,ⅠE和ⅡE期患者应以放射治疗为主要治疗手段。     

鼻咽非霍奇金淋巴瘤的临床与预后分析

目的 探讨鼻咽非霍奇金淋巴瘤的临床特点、治疗及国际预后指数(IPI)的应用价值。方法 回顾性分析1983—1997年间136例首程治疗的鼻咽非霍奇金淋巴瘤患者的临床资料。按照工作分类原则进行分类,高度恶性18例,中度恶性77例,低度恶性2例,未分类39例。根据Ann Arbor分期,Ⅰ期25例,Ⅱ期91例,Ⅲ期12例,Ⅳ期8例。Ⅰ期单纯放疗13例,综合治疗12例;Ⅱ期单纯放疗31例,综合治疗57例,Ⅲ和Ⅳ期以化疗为主。结果 5年和10年总生存率(OS)、癌症相关生存率(CSS)和无病生存率(OFS)分别为56．2％和48．3％,61．2％和58．0％,51．1％和46．5％。IPI为0,1及2～3分的5年CSS分别为70．9％、44．9％、30．0％(P=0．004)。Ⅰ期化放疗综合治疗和单纯放射治疗的5年CSS分别为82．2％和83．1％,10年CSS分别为82．2％和66．4％,差异无显著性(P=0．779)。Ⅱ期综合治疗和单纯放射治疗5年CSS分别为70．9％和46．0％,10年CSS分别为65．4％和46．0％,差异有显著性(P=0．04)。Cox多因素分析显示,影响预后的因素为Ann Arbor分期、B组症状和IPI。结论 IPI是判断原发于鼻咽非霍奇金淋巴瘤预后的重要指标,Ⅱ期鼻咽非霍奇金淋巴瘤应考虑综合治疗。     

Outcome of patients with non-Hodgkin's lymphoma of the stomach after gastrectomy: clinicopathologic study and reclassification according to the revised European-American lymphoma classification

Background. The best treatment for patients with non-Hodgkin's lymphoma (NHL) of the stomach is still uncertain. The revised European-American lymphoma (REAL) classification has helped to define new, potentially more appropriate classification schemes for gastric lymphomas. Methods. Fifty-one resected gastric lymphomas were reclassified according to the REAL classification, and the efficacy of multimodal treatment was examined retrospectively. The principal treatment plan consisted of: (1) surgical resection of the stomach with lymph node dissection, followed by (2) systemic chemotherapy, mainly using the cyclophosphamide/doxorubicin/vincristine/prednisone (CHOP) regimen. Results. According to the Ann Arbor classification, 27 patients had stage IE, 19 had stage IIE, and 5 had stage IV NHL. Using the REAL classification, we diagnosed diffuse large B-cell lymphoma (DLBL) in 23 patients, marginal zone B-cell (low-grade mucosa-associated lymphoid tissue [MALT]-type) lymphoma in 22, follicle center lymphoma in 4, mantle cell lymphoma in 1, and peripheral T-cell lymphoma in 1 patient. Nine of the 51 patients relapsed, and 8 patients with DLBL died of cancer. Survival rates at 5 years after surgery were 96.0% for stage IE, 83.3% for stage IIE, and 87.0% for all patients. Univariate analysis indicated that the tumor histology (according to the REAL classification), depth of invasion, degree of nodal involvement, Ann Arbor staging, and chemotherapy had an impact on patient outcome (P = 0.0018; P = 0.0002; P = 0.0308; P = 0.0016, and P = 0.0118, respectively). Conclusions. These data reveal that gastric NHL, especially of the low-grade MALT-type, often remains localized and has a good prognosis after surgery. The REAL classification was useful for classifying new categories of NHL, including the MALT-type, in the clinical setting, and for determining the optimal treatment modality for gastric NHL. Received: December 11, 2000 / Accepted: July 18, 2001     

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.     

原发性骨淋巴瘤临床特点及预后分析

目的 探讨原发性骨淋巴瘤(PBL)的临床特点、疗效及其与预后相关的因素.方法 对郑州大学第一附属医院2012年1月至2016年2月收治的19例PBL患者资料进行回顾性分析,探讨其临床病理特征及预后影响因素.结果19例患者中,男性11例,女性8例,中位年龄51岁;主要发病部位为股骨(5例)和脊柱(5例);8例行放化疗联合治疗,11例行单纯化疗;总有效率89.5%,包括完全缓解12例,部分缓解5例.2年总生存(OS)率、无进展生存(PFS)率分别为61.0%、48.3%.其中15例原发性骨弥漫大B细胞淋巴瘤(PB-DLBCL)患者2年OS率、PFS率分别为59.2%、45.5%.单因素分析显示淋巴瘤国际预后指数(IPI)评分≤2分、无软组织侵犯、初治完全缓解为OS及PFS的有利预后因素,美国东部肿瘤研究组(ECOG)评分≤2分为OS的有利预后因素,联合利妥昔单抗治疗为PFS的有利预后因素.多因素分析提示IPI评分是OS的独立预后因素,软组织侵犯是PFS的独立预后因素.结论 IPI评分≤2分及软组织未侵犯的PB-DLBCL患者预后较好.应根据局部软组织侵犯情况,探讨更合适的IPI评分模型.     

Blastic variant of mantle cell lymphoma: a rare but highly aggressive subtype.

The blastic variant (BV) form of mantle cell lymphoma (MCL) is considered to be a very aggressive subtype of non-Hodgkin's lymphoma (NHL). In order to determine its clinico-biological features and response to therapy we studied 33 patients (17%) out of 187 suffering from MCL who were diagnosed with a BV of MCL. Blastic variant was diagnosed according to histopathological patterns, immunophenotyping, and bcl1 gene rearrangement and/or cyclin D1 overexpression. Three patients initially diagnosed with large cell NHL were classified as BV. Patients received front-line therapy including CHOP-like regimen or CVP (n = 29), or chlorambucil (n = 4) and CHOP or ESAP as second-line therapy. High-dose intensification with stem cell transplantation (SCT) was performed in 11 cases (autoSCT, n = 8; alloSCT, n = 3). All but two patients were in complete remission (CR) at the time of transplant (CR1, n = 5; CR2, n = 4). Clinical and biological characteristics did not differ from those of the common form of MCL. The median age was 62 years (29-80), with a sex ratio (M/F) of 2.6:1. Of the 33 patients, 66% had extranodal site involvement, 85% had an Ann Arbor stage IV, and 82% had peripheral lymphadenopathy. Circulating lymphomatous cells were seen in 48% of cases. Twelve patients (36%) entered a CR1 with a median duration of 11 months. Fifteen patients (46%) failed to respond and rapidly died of progressive disease. Second-line therapy led to a 26% (6/23) CR2 rate. Nine patients relapsed after high-dose therapy. Twenty-two of the 33 patients (66%) died of refractory or progressive disease. Median overall survival (OS) time was 14.5 months for the 33 BV patients as compared to 53 months for the 154 patients with a common form of MCL, P <0.0001. In the univariate analysis, OS was influenced by age, extranodal site involvement, circulating lymphomatous cells, and international prognosis index (IPI). In the multivariate analysis, only IPI affected OS: patients with IPI > or =2 had 8 months median OS as compared to 36 months median OS for patients with IPI <2, P = 0.003. Blastic variant is one of the worst forms of NHL. An improved recognition of BV of MCL is required, particularly in high-grade CD5+ NHL using immunophenotyping and bcl1 molecular study. Standard therapy using anthracycline or even high-dose intensification produce poor results and an alternative treatment should be proposed to such patients.     

57例原发女性生殖系统淋巴瘤临床特征分析

背景与目的：原发女性生殖系统非霍奇金淋巴瘤（primary female genital system lymphoma，PFGSL）是女性生殖系统少见的恶性肿瘤，其发病率低，临床报道较少。探讨PFGSL的临床特征、诊治及预后。方法：回顾分析2008年1月—2019年12月期间于复旦大学附属肿瘤医院就诊的57例PFGSL患者的临床病理学资料。57例患者均符合PFGSL诊断标准，其中单一治疗组33例，综合治疗组24例。中位随访时间37.5个月（2.0~85.0个月）。病灶位于宫颈的40例（70.2%）、卵巢13例（22.8%）、子宫3例（5.3%）、阴道1例（1.8%）。按国际预后指数（International Prognostic Index，IPI）评分，低危组36例（63.2%），中危组21例（36.8%）。按淋巴瘤分期标准，ⅠE期36例（63.2%），ⅡE期19例（33.3%），ⅢE期2例（3.5%）。病理学类型为弥漫大B细胞淋巴瘤的患者有42例（73.7%）。结果：57例患者中，复发14例，复发率为24.6%（14/57），其中12例位于单一治疗组，2例位于综合治疗组。其中复发位于中枢神经系统者4例，眼1例；局部复发者4例，全身多处转移者5例。57例患者的3年总生存（overall survival，OS）率为82%，5年OS率为78%；3年无病生存（disease-free survival，DFS）率为77%，5年DFS率为71%。单一治疗组复发率为36.4%（12/33），3年OS率为72%，5年O率为64%；3年DFS率为67%，5年DFS率为57%。综合治疗组复发率仅为8.3%（2/24），3、5年OS率为95%；3、5年DFS率为为91%。多因素分析显示，患者的OS率及DFS率均与治疗方式明显相关（P＜0.05)。结论：原发女性生殖系统淋巴瘤发病率低，临床表现无特异性，极易造成误诊，妇科医师应提高对此病的认识，并参考放化疗科医师意见进行诊治。     

Characterization of peripheral T-cell lymphomas in a single North American institution by the WHO classification.

BACKGROUND: All peripheral T-cell lymphomas (PTCLs) diagnosed at a single institution were evaluated to determine the unique clinical features and outcome of specific entities and test the predictive validity of the International Prognostic Index (IPI). PATIENTS AND METHODS: Cases of PTCL seen at the British Columbia Cancer Agency between 1981 and 2000 were identified. Pathologic material was re-assessed and classified according to the WHO classification, and patients were staged and treated uniformly according to era-specific guidelines. In total, there were 199 patients with PTCL and the most common subtypes were peripheral T-cell lymphoma unspecified (PTCL-US) (59%), anaplastic large-cell lymphoma, systemic type (ALCL) (17%) and extranodal NK/T-cell lymphoma, nasal and nasal-type (NASAL) (9%). Most patients were treated with CHOP-type chemotherapy. RESULTS: Three distinct prognostic subgroups were notable on survival analysis: favorable (cutaneous ALCL), 5-year overall survival (OS) 78%; intermediate [PTCL, ALCL and angioimmunoblastic lymphoma (AILT)], 5-year OS 35-43%; unfavorable [NASAL and enteropathy-type T-cell lymphoma (ETTL)], 5-year OS 22-24%. Furthermore, in PTCL-US and ALCL clinical separation of patients into good risk (IPI 0,1) and poor risk (IPI > or =2) subsets was demonstrated. CONCLUSIONS: A large proportion of PTCL patients have poor risk disease and/or a histologically aggressive subtype with frequent relapse and unfavorable outcome. For these patients, treatment with CHOP chemotherapy is only minimally effective and new strategies need to be developed, an effort that will require a multi-institution international collaboration due to the rarity of most subtypes.