S. Luminari, L. Conte, N. Shokun, M. Civallero, G. Hapgood, M. Prince, E. Miranda, C. Chiattone, M. E. Cabrera, A. Pavlovsky, F. Hits, T. Skrypets, S. M. Rodriguez Pinilla, F. Francine, R. Advani, J. Vose, M. Federico
{"title":"淋巴结侵袭性t细胞淋巴瘤的临床结果和预后因素：来自t细胞项目2.0中登记的655例病例的见解","authors":"S. Luminari, L. Conte, N. Shokun, M. Civallero, G. Hapgood, M. Prince, E. Miranda, C. Chiattone, M. E. Cabrera, A. Pavlovsky, F. Hits, T. Skrypets, S. M. Rodriguez Pinilla, F. Francine, R. Advani, J. Vose, M. Federico","doi":"10.1002/hon.70094_388","DOIUrl":null,"url":null,"abstract":"<p>L. Conte, N. Shokun, M. Civallero, G. Hapgood, M. Prince, E. Miranda, C. Chiattone, M. E. Cabrera, A. Pavlovsky, F. Hits, T. Skrypets, S. M. Rodriguez Pinilla, F. Francine, R. Advani, and J. Vose equally contributing author.</p><p><b>Background:</b> According to the WHO classification of hematopoietic tissue tumors, the group of nodal aggressive T-cell lymphomas (TCLs) consists of PTCL-NOS, ALCL ALK-, AITL, and Nodal TCL with T-follicular helper phenotype. Despite advancements in therapeutic strategies, survival outcomes remain unfavorable. This study aims to evaluate the clinical characteristics, treatment patterns, and survival outcomes of patients diagnosed with nodal aggressive TCLs within the T-Cell Project 2.0, (clintrials.gov:03964480), an international prospective registry designed to improving the understanding of T-cell malignancies.</p><p><b>Methods:</b> A total of 655 patients diagnosed with nodal aggressive TCL between January 1, 2018, and June 30, 2023, were analyzed. Kaplan-Meier curves were used to estimate OS and PFS, while univariate and multivariate analyses identified prognostic factors.</p><p><b>Results:</b> The cohort included 312 patients with PTCL-NOS (47.6%), 172 with ALCL ALK- (26.3%), 158 with AITL (24.1%), and 13 with nodal TCL with TFH phenotype (1.9%). The median age was 60 yrs (19–93), with 59.7% males. The majority presented with stage III-IV (77.1%), 50.3% had B symptoms, 27.4% bone marrow involvement and 53.9% were EBV-positive.</p><p>The most frequent 1st line therapies were CHOEP (39.2%), CHOP/CHOP-like (30.8%) and BV-CHP. The CR rate was 49.0%, and PR 15.1%. Non-responders (NR) accounted for 35.9% of cases. The median follow-up was 26 months (95% CI: 22.4–29.6), with a 45.3% mortality rate.</p><p>The 2-yr OS and PFS rates were 50.8% and 36.1%, respectively. Patients achieving CR had significantly better survival outcomes, with a 2-yrs OS of 78.8% and PFS of 61.9%, while those with PR had a 2-yr OS of 34.8% and PFS of 21%. Of note, patients treated with BV-CHP showed a significantly better outcome (<i>p</i> < 0.001).</p><p>Univariate analysis identified age > 60 yrs (<i>p</i> < 0.001), stage III-IV (<i>p</i> < 0.001), ECOG ≥ 1 (<i>p</i> < 0.001), LDH > UNL (<i>p</i> < 0.001), hemoglobin < 12 g/dL (<i>p</i> < 0.001), platelets < 150 g/L (<i>p</i> < 0.001), albumin < 35 g/L (<i>p</i> = 0.01), histotype (ALCL- vs. PTCL-NOS or AITL: <i>p</i> < 0.001), IPI ≥ 2 (<i>p</i> < 0.001), and PIT ≥ 2 (<i>p</i> < 0.001) as significant negative prognostic factors for both OS and PFS.</p><p>In the multivariate analysis, histotype, age > 60 yrs, and stage III-IV emerged as independent prognostic factors for both OS and PFS (<i>p</i> < 0.001). Additionally, low PLT count was a significant independent predictor of worse PFS (<i>p</i> < 0.01).</p><p><b>Conclusions:</b> Nodal TCLs remain highly aggressive diseases with poor long-term survival. Patients with PCTL-NOS and AITL had similar outcome, inferior to ALCL ALK-. Response to f1 st therapy impacts survival, emphasizing the need for novel therapeutic strategies and risk-adapted treatment approaches. The identification of independent prognostic factors provides critical insights into risk stratification and tailored treatment planning. Further investigations into targeted and immunomodulatory therapies are warranted to improve outcomes in this high-risk population.</p><p><b>Research</b> <b>funding declaration:</b> no disclosure</p><p><b>Keywords:</b> aggressive T-cell non-Hodgkin lymphoma</p><p>No potential sources of conflict of interest.</p>","PeriodicalId":12882,"journal":{"name":"Hematological Oncology","volume":"43 S3","pages":""},"PeriodicalIF":3.3000,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/hon.70094_388","citationCount":"0","resultStr":"{\"title\":\"CLINICAL OUTCOMES AND PROGNOSTIC FACTORS IN NODAL AGGRESSIVE T-CELL LYMPHOMAS: INSIGHTS FROM 655 CASES REGISTERED IN THE T-CELL PROJECT 2.0\",\"authors\":\"S. Luminari, L. Conte, N. Shokun, M. Civallero, G. Hapgood, M. Prince, E. Miranda, C. Chiattone, M. E. Cabrera, A. Pavlovsky, F. Hits, T. Skrypets, S. M. Rodriguez Pinilla, F. 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引用次数: 0
摘要
L. Conte， N. Shokun， M. Civallero， G. Hapgood， M. Prince， E. Miranda， C. Chiattone， M. Cabrera， A. Pavlovsky， F. Hits， T. Skrypets， S. M. Rodriguez Pinilla， F. Francine， R. Advani，和J. Vose。背景：根据WHO对造血组织肿瘤的分类，淋巴结侵袭性t细胞淋巴瘤（TCLs）组包括PTCL-NOS、ALCL - ALK-、AITL和伴有t滤泡辅助表型的淋巴结TCL。尽管治疗策略取得了进步，但生存结果仍然不利。本研究旨在评估t细胞项目2.0 （clintrials.gov:03964480）中诊断为淋巴结侵袭性tcl患者的临床特征、治疗模式和生存结果，该项目是一个旨在提高对t细胞恶性肿瘤认识的国际前瞻性登记项目。方法：对2018年1月1日至2023年6月30日诊断为淋巴结侵袭性TCL的655例患者进行分析。Kaplan-Meier曲线用于估计OS和PFS，而单变量和多变量分析确定预后因素。结果：该队列包括312例PTCL-NOS患者（47.6%），172例ALCL患者（26.3%），158例AITL患者（24.1%），13例结节性TCL患者伴TFH表型（1.9%）。年龄中位数为60岁（19-93岁），男性占59.7%。多数为III-IV期（77.1%），50.3%有B型症状，27.4%骨髓受累，53.9% ebv阳性。最常见的一线治疗是CHOP（39.2%）、CHOP/CHOP-like（30.8%）和BV-CHP。CR为49.0%，PR为15.1%。无应答者（NR）占35.9%。中位随访时间为26个月（95% CI: 22.4-29.6），死亡率为45.3%。2年OS和PFS分别为50.8%和36.1%。达到CR的患者有明显更好的生存结果，2年OS为78.8%，PFS为61.9%，而PR患者的2年OS为34.8%，PFS为21%。值得注意的是，BV-CHP治疗的患者表现出明显更好的结果(p <；0.001)。单变量分析确定了年龄；60岁(p <；0.001)， III-IV期(p <；0.001), ECOG≥1 (p <；0.001), LDH >；l & p；0.001)，血红蛋白<；12 g/dL (p <；0.001)，血小板<；150克/升(p <；0.001)，白蛋白<；35 g/L (p = 0.01)，组织类型(ALCL- vs. PTCL-NOS或AITL: p <；0.001), IPI≥2 (p <；0.001), PIT≥2 (p <；0.001)是OS和PFS的显著负面预后因素。在多变量分析中，组织型、年龄>；60年，III-IV期成为OS和PFS的独立预后因素(p <；0.001)。此外，低PLT计数是PFS恶化的重要独立预测因子(p <；0.01)。结论：淋巴结性tcl仍然是高度侵袭性疾病，长期生存率较差。PCTL-NOS和AITL患者预后相似，低于ALCL - ALK-。对第一次治疗的反应影响生存，强调需要新的治疗策略和适应风险的治疗方法。独立预后因素的识别为风险分层和量身定制的治疗计划提供了关键的见解。有必要进一步研究靶向和免疫调节疗法，以改善这一高危人群的预后。研究经费声明：无披露关键词：侵袭性t细胞非霍奇金淋巴瘤潜在利益冲突来源
CLINICAL OUTCOMES AND PROGNOSTIC FACTORS IN NODAL AGGRESSIVE T-CELL LYMPHOMAS: INSIGHTS FROM 655 CASES REGISTERED IN THE T-CELL PROJECT 2.0
L. Conte, N. Shokun, M. Civallero, G. Hapgood, M. Prince, E. Miranda, C. Chiattone, M. E. Cabrera, A. Pavlovsky, F. Hits, T. Skrypets, S. M. Rodriguez Pinilla, F. Francine, R. Advani, and J. Vose equally contributing author.
Background: According to the WHO classification of hematopoietic tissue tumors, the group of nodal aggressive T-cell lymphomas (TCLs) consists of PTCL-NOS, ALCL ALK-, AITL, and Nodal TCL with T-follicular helper phenotype. Despite advancements in therapeutic strategies, survival outcomes remain unfavorable. This study aims to evaluate the clinical characteristics, treatment patterns, and survival outcomes of patients diagnosed with nodal aggressive TCLs within the T-Cell Project 2.0, (clintrials.gov:03964480), an international prospective registry designed to improving the understanding of T-cell malignancies.
Methods: A total of 655 patients diagnosed with nodal aggressive TCL between January 1, 2018, and June 30, 2023, were analyzed. Kaplan-Meier curves were used to estimate OS and PFS, while univariate and multivariate analyses identified prognostic factors.
Results: The cohort included 312 patients with PTCL-NOS (47.6%), 172 with ALCL ALK- (26.3%), 158 with AITL (24.1%), and 13 with nodal TCL with TFH phenotype (1.9%). The median age was 60 yrs (19–93), with 59.7% males. The majority presented with stage III-IV (77.1%), 50.3% had B symptoms, 27.4% bone marrow involvement and 53.9% were EBV-positive.
The most frequent 1st line therapies were CHOEP (39.2%), CHOP/CHOP-like (30.8%) and BV-CHP. The CR rate was 49.0%, and PR 15.1%. Non-responders (NR) accounted for 35.9% of cases. The median follow-up was 26 months (95% CI: 22.4–29.6), with a 45.3% mortality rate.
The 2-yr OS and PFS rates were 50.8% and 36.1%, respectively. Patients achieving CR had significantly better survival outcomes, with a 2-yrs OS of 78.8% and PFS of 61.9%, while those with PR had a 2-yr OS of 34.8% and PFS of 21%. Of note, patients treated with BV-CHP showed a significantly better outcome (p < 0.001).
Univariate analysis identified age > 60 yrs (p < 0.001), stage III-IV (p < 0.001), ECOG ≥ 1 (p < 0.001), LDH > UNL (p < 0.001), hemoglobin < 12 g/dL (p < 0.001), platelets < 150 g/L (p < 0.001), albumin < 35 g/L (p = 0.01), histotype (ALCL- vs. PTCL-NOS or AITL: p < 0.001), IPI ≥ 2 (p < 0.001), and PIT ≥ 2 (p < 0.001) as significant negative prognostic factors for both OS and PFS.
In the multivariate analysis, histotype, age > 60 yrs, and stage III-IV emerged as independent prognostic factors for both OS and PFS (p < 0.001). Additionally, low PLT count was a significant independent predictor of worse PFS (p < 0.01).
Conclusions: Nodal TCLs remain highly aggressive diseases with poor long-term survival. Patients with PCTL-NOS and AITL had similar outcome, inferior to ALCL ALK-. Response to f1 st therapy impacts survival, emphasizing the need for novel therapeutic strategies and risk-adapted treatment approaches. The identification of independent prognostic factors provides critical insights into risk stratification and tailored treatment planning. Further investigations into targeted and immunomodulatory therapies are warranted to improve outcomes in this high-risk population.
期刊介绍：
Hematological Oncology considers for publication articles dealing with experimental and clinical aspects of neoplastic diseases of the hemopoietic and lymphoid systems and relevant related matters. Translational studies applying basic science to clinical issues are particularly welcomed. Manuscripts dealing with the following areas are encouraged:
-Clinical practice and management of hematological neoplasia, including: acute and chronic leukemias, malignant lymphomas, myeloproliferative disorders
-Diagnostic investigations, including imaging and laboratory assays
-Epidemiology, pathology and pathobiology of hematological neoplasia of hematological diseases
-Therapeutic issues including Phase 1, 2 or 3 trials as well as allogeneic and autologous stem cell transplantation studies
-Aspects of the cell biology, molecular biology, molecular genetics and cytogenetics of normal or diseased hematopoeisis and lymphopoiesis, including stem cells and cytokines and other regulatory systems.
Concise, topical review material is welcomed, especially if it makes new concepts and ideas accessible to a wider community. Proposals for review material may be discussed with the Editor-in-Chief. Collections of case material and case reports will be considered only if they have broader scientific or clinical relevance.
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