Comparison of peripheral T-cell lymphomas and diffuse large B-cell lymphoma

BACKGROUND: Peripheral T-cell lymphomas (PTCLs) are a biologically heterogeneous subgroup of lymphomas with poor prognosis. In this study, the authors analyzed the clinical behaviors of PTCLs and diffuse large B-cell lymphoma (DLBCL). METHODS: The authors compared the characteristics and outcomes of 59 patients with PTCLs, including 33 angioimmunoblastic T-cell lymphomas and 26 unspecified peripheral T-cell lymphomas, with the characteristics and outcomes of 193 patients with DLBCLs who were treated in the era before rituximab. RESULTS: Based on the clinical characteristics, elevated lactate dehydrogenase (LDH), poor PS, advanced stage, higher International Prognostic Index score, and B symptoms were more common in patients with PTCLs, and bulky mass was more common in patients with DLBCL. The rates of complete response (CR) or an unconfirmed CR (CRu) were higher in patients with DLBCL (72%) than in patients with PTCLs (56%; P = .03). The 5-year overall survival (OS), progression-free survival (PFS), and disease-free survival (DFS) rates were 31%, 26%, and 47%, respectively, in patients with PTCLs and 59%, 55%, and 73%, respectively, in patients with DLBCL (P = .001, P < .001, and P = .003, respectively). Although multivariate analysis identified several risk factors that were significant in PTCLs, but not in DLBCLs, for the CR/CRu, OS, PFS, and DFS rates, the immunophenotype was not identified as a risk factor. CONCLUSIONS: The poor response and survival of patients who had PTCLs, compared with patients who had DLBCL, was caused by numerous initial risk factors. T-cell phenotype itself did not appear to have a significant impact on either response or survival.     

Increasing incidence of non-Hodgkin's lymphoma in Canada, 1970-1996: age-period-cohort analysis

Previous studies have shown that the incidence of non-Hodgkin's lymphoma (NHL) has increased in many parts of the world in recent decades. Using data obtained from the Canadian Cancer Registry, the present study examined time trends in NHL incidence in Canada between 1970 and 1996 and the effects of age, period of diagnosis and birth cohort on incidence patterns for each sex separately. Results showed that overall age-adjusted incidence rates increased substantially, from 7.3 and 5.2 per 100,000 in 1970-1971 to 14.0 and 10.0 per 100,000 in 1995-1996 in males and females, respectively. Diffuse lymphoma was the major histological subtype, accounting for approximately 76% of NHL cases over the 27-year period. The data suggest that period effects have played a major role, although birth cohort effects may also have been involved. Sex-specific patterns of the incidence were similar over the time period of diagnosis but were distinct among recent birth cohorts. In conclusion, there is in fact a marked increase in NHL in Canada which cannot be explained in terms of improvements in diagnosis, changes in NHL classification and the increase in AIDS-associated NHL alone. The birth cohort effect in NHL suggests that changes in risk factors may have contributed to the observed increase.     

我国皮肤T细胞淋巴瘤发病情况分析

目的:通过回顾性数据分析,探讨皮肤T细胞淋巴瘤在中国的发病情况。方法:通过检索公开发表的有关淋巴瘤和皮肤T细胞淋巴瘤的发病情况研究文献,应用聚类分析法对符合纳入标准的文献进行纳入并统计其中皮肤T细胞淋巴瘤的发病率。经过汇总整理与《中国淋巴瘤亚型分布国内多中心性病例10 002例分析》进行对比。结果:本研究共纳入17篇文献,涉及13个省市自治区,共计16 885例患者。分析结果显示,中国皮肤T细胞淋巴瘤从1993年到2017年发病率约为0.69/105,与《中国淋巴瘤亚型分布国内多中心性病例10 002例分析》中的结果一致,与国外文献报道中美国皮肤T细胞淋巴瘤发病率0.64/105也基本一致。结论:皮肤T细胞淋巴瘤在中国和有关国家都是发病率很低的一种恶性肿瘤,属于WHO定义的罕见病。     

Racial differences in the presentation and outcomes of diffuse large B‐cell lymphoma in the United States

BACKGROUND:

Diffuse large B‐cell lymphoma (DLBCL) is often cured with standard chemoimmunotherapy, but there is great heterogeneity in presentation and outcomes.

METHODS:

By using Surveillance, Epidemiology, and End Results (SEER) data from 13 registries across the United States, the authors examined differences in incidence and survival for DLBCL by race. International Classification of Diseases for Oncology, third edition histology codes 9678, 9679, 9680, and 9684 were used to identify cases.

RESULTS:

From 1992 to 2007, 38,522 cases of DLBCL were recorded in SEER. Sixty‐five percent of black patients compared with 37% of white patients presented at age ≤60 years, 52% of blacks compared with 44% of whites presented with stage III/IV disease, and 31% of black versus 24% of white patients presented with B symptoms (all P < .001). Although survival improved by era of diagnosis for all races (log rank P < .001), 2‐year relative survival rates were better for women than men (61% vs 58%, P < .001) and white than black patients (60% vs 50%, P < .001). Black race, male sex, age at diagnosis >60, advanced stage, and B symptoms at diagnosis were predictors of worse survival (P < .001).

CONCLUSIONS:

Black patients with DLBCL in the United States present at younger age, more advanced stage, and have inferior survival. Epidemiological studies that examine the biological variants of DLBCL in concert with race are needed to elucidate the etiology of these disparities. Cancer 2011;. © 2010 American Cancer Society.     

Trimodal age‐specific incidence patterns for Burkitt lymphoma in the United States, 1973–2005

Burkitt lymphoma (BL) is a unique B‐cell non‐Hodgkin lymphoma with 3 established clinical‐epidemiological variants: endemic, sporadic and AIDS‐related BL. BL variants show characteristic dysregulation of MYC gene, but the causes of MYC dysregulation or BL arising at different ages are poorly understood. Therefore, we examined population‐based BL incidence patterns in the United States to determine age‐related risk. BL case and population data were obtained from the NCI's Surveillance, Epidemiology and End Results Databases (1973–2005). Standard cross‐sectional age‐standardized and age‐specific incidence rates were stratified by sex and race and supplemented with age–period–cohort models. We analyzed 3,058 BL cases diagnosed during 1,160,300,297 person‐years of observation. Age‐standardized incidence rates rose 6.8% per year (95% CI 4.5–9.1) for males and 7.1% (95% CI 3.2–11.1) for females during the study period. The rate among males was 3.2 times that among females, and among Whites 1.3 times that among Blacks. Male‐to‐female incidence rate ratios did not differ by race, but were 4.2 for pediatric (0–19 years), 4.1 for adult (20–59 years) and 2.0 for geriatric (≥60 years) BL. Cross‐sectional age‐specific rates showed 2 separate peaks among males and females, near ages 10 and 75 years, and a 3rd peak near age 40 years among males. The tri/bimodal incidence pattern was present in sensitivity analyses excluding registries with many HIV/AIDS cases and in period‐specific, cohort‐specific analyses. To our knowledge, tri/bimodal incidence patterns have not previously been reported for BL. Trimodal/bimodal BL suggests heterogeneity in etiology or biology of BL diagnosed at different ages in males and females. © 2009 UICC.     

'Normal counterparts' of nodal peripheral T-cell lymphoma

Peripheral T‐cell lymphomas (PTCL) have been difficult to classify. A homogeneous principle of classification is still lacking, partly because lymph node compartments containing functionally distinct T‐cell subsets have not been identified. A correlation to differentiated T‐cell subsets, as CD4⁺ or CD8⁺ cells as well as cytotoxic populations has not revealed clinically meaningful entities. Upon antigen encounter, mature T‐cells pass through distinct stages characterized by their surface molecule expression. Naïve T‐cells are CD45RA⁺/CD45R0^?/CD27⁺/CCR7⁺, however, after antigen contact CD45RA expression is replaced by CD45R0. They differentiate to central memory cells, which retain CD27 and CCR7, or to effector‐memory cells, which loose expression of both molecules depending on the strength of the antigen interaction. Immunohistological analysis of PTCL showed an effector or effector‐memory cell phenotype (CD45RA^?/CD45R0⁺/CD27^?) for both angioimmunoblastic T‐cell lymphoma (AILT) and anaplastic large cell lymphoma (ALCL), but different cytotoxic and activation markers expressed by these tumours. A subset of CD4⁺ PTCL‐not otherwise specified (PTCL‐NOS) may correspond to a central memory cell phenotype (CD45RA^?/CD45R0⁺/CD27⁺). Thus, a correlation of PTCL to stages of differentiation, rather than to the direction of differentiation, may reveal homogeneous categories. A comparison between the lymphomas and their normal counterparts maycontribute to the understanding of the underlying transformation mechanisms. Copyright © 2006 John Wiley & Sons, Ltd.     

Increasing incidence and continued dismal outcome of primary central nervous system lymphoma in Norway 1989-2003 : time trends in a 15-year national survey

BACKGROUND: The incidence of primary central nervous system lymphoma (PCNSL) appears to be increasing in some countries, whereas it is stable in others. Many reports the last decades have suggested that there have been improvements in the treatment of PCNSL. The objective of this study was to analyze time trends in the incidence, clinical features, histologic diagnosis, treatment, and outcome of nonacquired immunodeficiency syndrome (non-AIDS) PCNSL in Norway from 1989 to 2003. METHODS: Patients were identified by a chart review of all patients who had a recorded diagnosis of PCNSL from 1989 to 2003 in The Norwegian Cancer Registry. The histologic and cytologic material from each patient was re-examined by pathologists. Time trends were analyzed according to year of diagnosis grouped into 3 5-year periods: 1989-1993, 1994-1998, and 1999-2003. RESULTS: There were 98 patients who had confirmed, newly diagnosed non-AIDS PCNSL in Norway from 1989 to 2003. The incidence rate increased during the consecutive 5-year periods from 0.89 per million during 1989 to 1993, to 1.74 per million during 1994 to 1998, and to 1.82 per million during 1999 to 2003 (P = .013). Diagnostic delay and overall survival did not improve with time. Survival decreased from 1999 to 2003 compared with survival from 1994 to 1998, which was explained in part by reduced performance status and fewer patients receiving combined chemotherapy and radiotherapy during 1999 to 2003. In multivariate analysis, age 相似文献   

CT features of peripheral T-cell lymphoma in the gastrointestinal tract in Chinese population and literature review

Introduction: The aim of the study was to retrospectively investigate the CT features in peripheral T‐cell lymphoma (PTCL) of the gastrointestinal tract in the Chinese population. Methods: Computed tomography scans of 15 histopathologically proven cases of PTCL involving the gastrointestinal tract were retrospectively reviewed for characteristics such as sites, multiplicity, morphological features, the pattern and degree of contrast enhancement, lymphadenopathy, involvement of other organs and complications such as perforation, intussusceptions, ascites and so on. By reviewing the literature, CT findings of PTCL involving the gastrointestinal tract were compared with that involved by B‐cell lymphoma. Results: PTCLs involved the stomach and intestine in six and nine patients, respectively. Multiplicity was seen in seven patients, and solitary involvement was seen in eight. At CT, wall thickening was the predominant finding in all cases with an exception of one intestinal PTCL case presented as polypoid mass. Among the 14 patients, the gastric or bowel wall thickening was mild (<10 mm) in three, moderate (10–20 mm) in 10 and severe (>20 mm) in one. Nine cases demonstrated mild homogeneous enhancement, whereas six showed mild heterogeneous enhancement. Lymphadenopathy was present in eight patients, five of which were non‐bulky (diameter <5 cm) and diffuse type and the rest (three) were non‐bulky and localised type. Other organs were involved in four patients. Perforation as complication was evident in one gastric and five intestinal lymphomas (55.6%). Among the nine intestinal PTCLs, seven of the patients were male (77.9%) and the rest (two) were female with a median age of 37.1 years old. Intestinal PTCLs predominantly involved colon (n = 5). Other sites of involvement were ileum (n = 1), ileocaecum (n = 1), ileum and ileocaecum (n = 1) and entire bowel segment from distal ileum to transverse colon (n = 1). Conclusion: PTCLs have some distinguishing radiological features from B‐cell type gastrointestinal lymphomas as mild or moderate gastric or bowel wall thickening and higher incidence of perforation with multiplicity. In China, intestinal PTCLs are not usually associated with coeliac disease and commonly present in a young male population with colon being the most frequent site of involvement.     

T- and B-cell clonality and frequency of human herpes viruses-6, -8 and Epstein Barr virus in angioimmunoblastic T-cell lymphoma

Angioimmunoblastic T-cell lymphoma (T-AIL) is a peripheral T-cell lymphoma of unknown etiology. Previous clonality studies have shown a heterogeneous composition of this disease with varying restrictions of B- and T-cell populations in the tumour. For the first time in a single study and in the same pathological materials, we have analysed, lymphoid cell clonality and occurrence of human herpes viruses and Epstein Barr virus. Of 18 cases 12 (66.6%) had clonal T- and three (16.6%) had clonal B-cells. Presence of the lymphotropic viral genome of HHV6 was detected in four of 18 lymph node biopsies from T-AIL patients (22%), all were TCRgamma clonal. No HHV8 were found. Epstein Barr genome was found in 40% of cases. There was no significant association between T-cell clonality and HHV-6 or EBV infection, or between B-cell clonality and any virus infection. We conclude that T-AIL is a biologically and clinically heterogeneous entity whose true nature remains to be clarified.     

T-CELL RECEPTOR GENE REARRANGEMENT ANALYSIS IN THE PRIMARY CUTANEOUS T-CELL LYMPHOMA

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Intestinal T‐cell lymphoma with enteropathy‐associated T‐cell lymphoma‐like features arising in the setting of adult autoimmune enteropathy

Enteropathy‐associated T‐cell lymphoma is regarded as a dismal, late complication of coeliac disease, though a single case of T‐cell lymphoma with such features arising in the setting of autoimmune enteropathy of the adult has been reported to date. We aim to describe the case of a 41‐year‐old woman complaining of severe malabsorption syndrome, who was diagnosed with autoimmune enteropathy based on the presence of flat intestinal mucosa unresponsive to any dietary restriction and positivity for enterocyte autoantibodies. Steroid therapy led to a complete recovery of both mucosal and clinical findings over 12 years, when disease relapse was accompanied by the appearance of monoclonal rearrangement of T‐cell receptor‐γ and peculiar T‐cell phenotypic abnormalities, leading to a rapid transition to an overt T‐cell lymphoma with features of the enteropathy‐associated subtype. Despite intensive treatment, the patient developed cerebral metastasis and died 9 months later. Our case enhances the concept of enteropathy‐associated T‐cell lymphoma as a disease that may arise in the setting of enteropathies other than coeliac disease, thus representing a heterogeneous entity. Moreover, our observations support the need of a close follow‐up of these patients, coupled with comprehensive characterization of mucosal biopsies.     

Increasing incidence of differentiated thyroid cancer in the United States, 1988–2005

BACKGROUND:

Studies have reported an increasing incidence of thyroid cancer since 1980. One possible explanation for this trend is increased detection through more widespread and aggressive use of ultrasound and image‐guided biopsy. Increases resulting from increased detection are most likely to involve small primary tumors rather than larger tumors, which often present as palpable thyroid masses. The objective of the current study was to investigate the trends in increasing incidence of differentiated (papillary and follicular) thyroid cancer by size, age, race, and sex.

METHODS:

Cases of differentiated thyroid cancer (1988‐2005) were analyzed using the National Cancer Institute's Surveillance Epidemiology and End Results (SEER) dataset. Trends in incidence rates of papillary and follicular cancer, race, age, sex, primary tumor size (<1.0 cm, 1.0‐2.9 cm, 3.0‐3.9 cm, and >4 cm), and SEER stage (localized, regional, distant) were analyzed using joinpoint regression and reported as the annual percentage change (APC).

RESULTS:

Incidence rates increased for all sizes of tumors. Among men and women of all ages, the highest rate of increase was for primary tumors <1.0 cm among men (1997‐2005: APC, 9.9) and women (1988‐2005: APC, 8.6). Trends were similar between whites and blacks. Significant increases also were observed for tumors ≥4 cm among men (1988‐2005: APC, 3.7) and women (1988‐2005: APC, 5.70) and for distant SEER stage disease among men (APC, 3.7) and women (APC, 2.3).

CONCLUSIONS:

The incidence rates of differentiated thyroid cancers of all sizes increased between 1988 and 2005 in both men and women. The increased incidence across all tumor sizes suggested that increased diagnostic scrutiny is not the sole explanation. Other explanations, including environmental influences and molecular pathways, should be investigated. Cancer 2009. © 2009 American Cancer Society.     

Acute promyelocytic leukemia: A population‐based study on incidence and survival in the United States, 1975‐2008

BACKGROUND:

With the introduction of all‐trans retinoic acid and arsenic trioxide, the management of acute promyelocytic leukemia (APL) has changed dramatically. We performed a population‐based study of APL in the United States to determine its incidence and relative survival (RS) during a 34‐year period.

METHODS:

We identified 1397 patients diagnosed with APL between 1975 and 2008 in the Surveillance, Epidemiology, and End Results database. Patients were categorized into 4 age groups and 3 calendar periods. As a comparison, we also reviewed the outcome of APL patients treated at our institution during approximately the same time interval.

RESULTS:

The incidences of APL increased with time period and patient age. Short‐ and long‐term RS improved with each calendar period, with the greatest improvement occurring between 1991 and 1999; 5‐year RS rates were 0.18 for patients diagnosed in 1975‐1990, 0.52 in 1991‐1999, and 0.64 in 2000‐2008. Age was an important predictor of survival. For example, the 5‐year RS rate in patients diagnosed in 2000‐2008 was 0.38 for patients aged ≥60 years and 0.73 and 0.75 for patients aged <20 years and 20‐39 years, respectively. Similar treads of improvements in the survival were observed in APL patients treated at our institution.

CONCLUSIONS:

The incidence of APL has increased, especially in the last decade. Clinical outcome improved remarkably in patients with APL diagnosed from 1991 to 1999, mainly because of the increased use of all‐trans retinoic acid. Cancer 2012;. © 2012 American Cancer Society.     

Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975‐2005

BACKGROUND:

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents aged <20 years; its etiology remains largely unknown. It is believed that embryonal (ERMS) and alveolar rhabdomyosarcoma (ARMS), the most common subtypes, arise through distinct biologic mechanisms. The authors of this report evaluated incidence and survival trends by RMS demographic subgroups to inform future etiologic hypotheses.

METHODS:

Incidence and survival trends in RMS among children and adolescents aged <20 years were analyzed using data from the Surveillance, Epidemiology, and End Results Program. Frequencies, age‐adjusted incidence and survival rates, and joinpoint regression results, including annual percentage change (APC) and 95% confidence interval (CI), were calculated.

RESULTS:

Between 1975 and 2005, the incidence of ERMS was stable, whereas a significant increase in the incidence of ARMS was observed (APC, 4.20%; 95%CI, 2.60%‐5.82%). This trend may have been attributable in part to shifts in diagnosis, because a significant negative trend in RMS, not otherwise specified was observed concurrently. A bimodal age peak for ERMS was observed, with the second, smaller peak in adolescence noted for males only; ARMS incidence did not vary by age or sex. Five‐year survival rates for RMS and ERMS increased during the period from 1976 to 1980 (52.7% and 60.9%, respectively) to the period from 1996 to 2000 (61.8% and 73.4%, respectively), whereas there was little improvement for ARMS (40.1% and 47.8%, respectively).

CONCLUSIONS:

Observed differences in incidence and survival for 2 major RMS subtypes across sex and age subgroups further supported the hypothesis that there are unique underlying etiologies for these tumors. Exploration of these differences presents an opportunity to increase current knowledge of RMS. Cancer 2009. © 2009 American Cancer Society.     

FDA drug approval summary: nelarabine (Arranon) for the treatment of T-cell lymphoblastic leukemia/lymphoma

PURPOSE: To describe the clinical trials leading to U.S. Food and Drug Administration (FDA) approval of nelarabine (Arranon), a new purine analogue, for the treatment of patients with T-cell acute lymphoblastic leukemia (T-ALL) and T-cell lymphoblastic lymphoma (T-LBL) whose disease has not responded to or has relapsed following treatment with at least two chemotherapy regimens. EXPERIMENTAL DESIGN: Two phase II trials, one conducted in pediatric patients and the other in adult patients, were reviewed. Patients were in their first or subsequent relapse and/or were refractory to first-line therapy. The dose and schedule of i.v. nelarabine in the pediatric and adult studies were 650 mg/m2 per day daily for 5 days and 1,500 mg/m2 i.v. on days 1, 3, and 5, respectively. Treatments were repeated every 21 days. Study endpoints were the rates of complete response (CR) and CR with incomplete hematologic or bone marrow recovery (CR*). RESULTS: The pediatric efficacy population consisted of 39 patients who had relapsed after, or had been refractory to, two or more induction regimens. CR to nelarabine treatment was observed in five patients (13%) and CR+CR* was observed in nine patients (23%). The adult efficacy population consisted of 28 patients. CR to nelarabine treatment was observed in five patients (18%) and CR+CR* was observed in six patients (21%). Neurologic toxicity was dose limiting for both pediatric and adult patients. Other severe toxicities included hematologic, hepatic, and metabolic laboratory abnormalities in pediatric patients and gastrointestinal and pulmonary toxicities in adults. CONCLUSIONS: On October 28, 2005, the FDA granted accelerated approval for nelarabine for treatment of patients with relapsed or refractory T-ALL/T-LBL after at least two prior regimens. This use is based on the induction of CR. The applicant will conduct postmarketing clinical trials to demonstrate clinical benefit, for example, survival prolongation.     

皮肤T淋巴细胞瘤的研究进展

随着对皮肤T细胞淋巴瘤(CTCL)病理发生机制研究的深入以及肿瘤治疗技术的进步,近年来对CTCL诊断及治疗方法也有了一定的进展.早期CTCL首选局部治疗及免疫治疗.全身皮肤电子束治疗、靶向治疗及化疗、联合治疗、维持治疗是进展期CTCL的主要治疗手段.近年来新的药物及治疗方法不断涌现,本文对皮肤T细胞淋巴瘤的临床特点及其...