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1692 The Number of Extranodal Sites Matters! a Proposal for a Feasible Prognostic Model in Nodal PTCL. a Collaborative Study grupo De Estudio Latino-Americano De Linfoproliferativos (GELL) & T-Cell Brazil Project (TCBP)

Program: Oral and Poster Abstracts
Session: 625. T Cell, NK Cell, or NK/T Cell Lymphomas: Clinical and Epidemiological: Poster I
Hematology Disease Topics & Pathways:
Adult, Study Population, Human
Saturday, December 7, 2024, 5:30 PM-7:30 PM

Thais Fischer, MD1*, Eliana Miranda, PhD, MEd2*, Juliana Pereira, MD, PhD3, Guilherme Duffles, MD4*, Jamila Vaz Tavarez, MD5*, Nelson S Castro, MD6*, Rodrigo Santucci7*, Danielle Leão Cordeiro De Farias, MD, MSc, MBA8, Sergio Brasil, MD9*, Claudio G Macedo, MD10*, Carolina Colaco, MD11*, Renata Lyrio R Baptista, MD12,13*, Karin Z Cecyn, MD14*, Davimar Bortucchi, MD, PhD15*, Gilnara Fontinele Silva, MD, MSc16*, Samir Nabhan, MD17*, Patricia Giacon, MD18*, Rony Schaffel, MD, PhD19, Natalia Pin Chuen Zing, MD20,21*, Frederico Lisboa Nogueira, MD22, Ademar Dantas Cunha, PhD23, Diego Vila Cle24*, Jose D Tadeu, MD25*, Vera Figueiredo, MD26*, Mateus Dalló Dal Pont, MD27*, Rafael Gaiolla, MD, PhD28*, Nelson Hamerschlak, MD, PhD29, Eduardo Flavio Oliveira Ribeiro30*, Abrahão Elias Hallack Neto, MD, PhD31*, Maria Almeida Dias, MD32*, Yung Gonzaga, MD33*, Yana S Rabelo, MD34*, Larissa Lane Teixeira, MD35*, Guilherme Fleury Perini, MD36,37, Milene Matedi, MD38*, Priscilla Cury39*, Henry Idrobo, MD40*, Denisse Castro, MD41, Brady Beltran, MD41*, Daniel J Enriquez, MD42, Jule F Vasquez, MD43, Claudia Roche, MD44*, Daniel Artiles, MD45*, Fabiola Valverte46*, Luis Mario Villela Martinez47*, Ana Carolina Oliver, MD48, Laura Korin, MD49*, Camila Peña, MD50, Macarena Roa, MD51*, Maria Alejandra Torres, MD52*, Seisha Alana von Glasenapp, MD53, Alfredo Quiroz, MD54*, Cesar Samanez-Figari, MD55*, Rosa Oliday Rios Jimenez56*, Sally Paredes, MD41*, Casey Bermack, MD57, Kelly Meza, MD58*, Bryan Valcarcel, MD, MPH59, Carmino A Souza, MD60*, Luis Malpica, MD61* and Carlos Chiattone, MD, PhD62

1AC Camargo Cancer Center, Sao Paulo, AC, Brazil
2Centro de Hematologia e Hemoterapia (HEMOCENTRO), Universidade Estadual de Campinas (UNICAMP), Campinas, Brazil
3University of São Paulo, Department of Hematology, Hemotherapy & Cell Therapy, University of São Paulo, São Paulo, São Paulo, Brazil
4Centro de Hematologia e Hemoterapia, Universidade Estadual de Campinas (UNICAMP), Campinas, Brazil
5Ophir Loyola Hospital, Belem, Brazil
6Hospital de Cancer de Barretos, Barretos, São Paulo, BRA
7HEMOMED, Sao Paulo, AM, BRA
8Beneficencia Portuguesa Hospital, Sao Paulo, Brazil
9Santa Casa Medical School of Sao Paulo, Sao Paulo, Brazil
10CECAN, Natal, Brazil
11Liga Contra o Cancer CECAN, Natal, Brazil
12Instituto D'Or de Pesquisa e Ensino, Rio de Janeiro, Brazil
13State University of Rio de Janeiro, Rio De Janeiro, Brazil
14Federal University of Sao Paulo, Sao Paulo, Brazil
15ABC Medical School, Santo Andre, Brazil
16Aldenora Bello Hospital, Sao Luis, Brazil
17Federal University of Paraná, Parana, Brazil
18Santa Marcelina Hospital, Sao Paulo, Brazil
19HUCFF / UFRJ, Rio de Janeiro, Brazil
20Prevent Senior, SAO PAULO, Brazil
21Hospital Beneficencia Portuguesa de SP, Sao Paulo, Brazil
22Luxemburgo Hospital, Belo Horizonte, Brazil
23Hospital do Cancer de Cascavel - UOPECCAN (Uniao Oeste Paranaense de Estudos e Combate ao Cancer), Cascavel, Brazil
24USP Ribeirão, Ribeirao Preto, Brazil
25FM Campos, Campos dos Goytacases, Brazil
26Servidor Publico Estadual de Sao Paulo, Sao Paulo, Brazil
27Centro de Pesquisas Oncologicas - CEPON, Santa Catarina, Brazil
28Hospital Das Clínicas UNESP, Botucatu, Brazil
29Departamento de Oncologia e Hematologia, Hospital Israelita Albert Einstein, Sao Paulo, Brazil
30Curitiba, Brazil; Department of Hematology, Santa Lucia Hospital - Brasilia, DF, Brazil, Brasilia, Brazil
31Juiz de Fora Federal University, Juiz de Fora, Brazil
32Federal da Bahia Hospital, Salvador, Brazil
33Cancer National Institute, Rio De Janeiro, Brazil
34Goias Federal University, Goias, Brazil
35Hospital Israelita Abert EInstein, São Paulo, BRA
36Hospital Paulistano, São Paulo, Brazil
37Department of Medical Oncology, Hospital Israelita Albert Einstein, São Paulo, Brazil
38Santa Casa de Belo Horizonte, Belo Horizonte, Brazil
39Clinica São Germano, São Paulo, Brazil
40Universidad del Valle del Cauca, Cali, COL
41Hospital Edgardo Rebagliati Martins, Lima, Peru
42Departamento de Oncologia Medica, Instituto Nacional de Enfermedades Neoplasicas, Lima, Peru
43Instituto Nacional de Enfermedades Neoplasicas, Lima, Peru
44Hospital Armando Milan Castro, Vila Clara, Cuba
45Hospital Arnoldo Milan Castro, Vila Clara, Cuba
46INCAN, Mexico, Mexico
47Mexico, Mexico, NM, MEX
48Hospital Britanico de Montevideo, Montevideo, Uruguay
49CABA- Alexander Fleming Institute, Olivos, Argentina
50Hospital del Salvador, Santiago, Chile
51Hospital Del Salvador, Santiago, Chile
52Santa Sofia Clinic, Caracas, Venezuela (Bolivarian Republic of)
53Instituo de Previsión Social, Caracas, Venezuela (Bolivarian Republic of)
54Departamento de Hematología, Hospital Central Instituto de Previsión Social, Asunción, Paraguay
55San Borja, Lima, Peru
56Hospital Clínico Quirúrgico Hermanos Amejeiras, La Habana, Cuba
57University of Texas MD Anderson Cancer Center, Houston, TX
58Baylor College of Medicine, Houston, TX
59Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Houston, TX
60University of Campinas, São Paulo, Brazil
61Department of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center, Houston, TX
62Santa Casa de São Paulo. São Paulo – Brazil, São Paulo, Brazil

Introduction

Peripheral T cell lymphoma (PTCL) accounts for 10-15% of all non-Hodgkin lymphomas. As previously described by our group (Fischer et al, 2023 ASH Meeting), Latin America has a distinct epidemiological PTCL distribution, with a high frequency of adult T cell leukemia/lymphoma and extranodal NKT cell lymphoma cases, likely influenced by distinct genetic profiles and viral epidemiology. Survival rates have not improved over the past 20 years, with an estimated OS of 40% at 3 years. Treatment advances have also been limited, except for BV-CHP which access is limited in low- and middle-income countries. Given the above, it is crucial to understand the risk factors related to poor outcomes in PTCL. The IPI, which includes extranodal (EN) site as a variable, has been validated for PTCL. However, the specific impact of EN involvement in nodal PTCL (such as PTCL-NOS, AITL, ALCL ALK+/ALK-) and its biological implications remain unclear. Simplification could improve the reproducibility and applicability of these models, especially in low- and middle-income countries.

Objective

We aim to evaluate the number of EN sites in nodal PTCL lymphomas as a risk factor or surrogate for outcomes such as OS and PFS in a Latin America cohort.

Methodology

We compiled data from patients (pts) aged ≥18 years with newly diagnosed nodal PTCL-NOS, AITL and ALCL ALK+/ALK-, from the retrospective registry of the Grupo de Estudio Latinoamericano de Linfoproliferativos (n=339, 2000-2023), the T-cell Brazil Project (n=427, 2015-2022, ambispective). Clinical information included coded patient and site identifiers, gender, age, date and site of diagnostic biopsy, sites of disease, EN (number) and Ann Arbor stage. Initial therapy and response; details on response and relapse rates; subsequent lines of therapy and vital status were also recorded. Our primary endpoints were OS and PFS. REDcap Platform (by Vanderbilt) has been used to collect and store data and for descriptive analysis the IBM-SPSS version 24 was applied. Kaplan-Meier method estimated the OS, whereas Log-Rank tests to compare its curves. This trial is registered at Clinical trials (NCT03207789).

Results

We enrolled 766 pts (427 TCBP; 339 GELL). We grouped pts according to the number of EN involvement: No EN (n=383); one EN (EN1 n=168) and ≥2 EN involvement (EN2 n=215). In the entire cohort, there was a slight male predominance (61%); median age 56 years (range 15-92 y/o); 74% stage III/IV; 69% IPI ≥2. Most frequently histology was PTCL-NOS (60%), followed by ALCL ALK- (19%) and AITL (12%). The majority had B symptoms (61%) and elevated LDH (55%). CHOEP was the most frequent chemotherapy used (47%), followed by CHOP (34%). Transplant was performed as consolidation in only 16% of the cases. Almost half of patients achieved complete response (CR) after first line (47%), although 33% relapsed. Clinical characteristics were similar between No EN, EN1 and EN2 groups except for stage III/IV (58% vs 79% vs 96%; p<.0001); IPI ≥2 (58% vs 59% vs 99%; p<.0001); ECOG>1 (58% vs 92% vs 99%; p<.0001); bone marrow involvement (16% vs 24% vs 63%%; p <.0001), elevated LDH (54% vs 47% vs 61%; p=.03) and albumin <3.5 g/dL (33% vs 38% vs 58%; p <.0001), respectively. As previously reported, better OS and PFS were seen in ALCL ALK+ compared to ALK- and PTCL-NOS. Among No EN, EN1 and EN2 groups, the CR rates after 1st line (54% vs 46% vs 37%, p=0.002), 60-month OS (45% vs. 44% vs. 27%, p <.0001) and PFS (31% vs. 34% vs. 18%; p <.0001) were inferior in the EN2 group, suggesting the number of EN sites involved assessed by CT and PET-CT matters and may be a feasible clinical surrogate for outcomes in this patient population.

Conclusion

PTCL remains an unmet medical need due to suboptimal treatment responses and outcomes. Thus, new biomarkers are essential to better stratify patients’ risk for PTCL mortality. In this large cohort of PTCL patients, we found the number of EN involvement as a useful and practical prognostic tool to predict response to therapy and outcomes. EN2 was associated to inferior response rates and shorter OS and PFS. Our findings indicate distinct biological characteristics in EN PTCL presentation that merit further molecular investigation, potentially enhancing tailored therapies. Registries are essential given the rarity and poor prognosis associated with these diseases as well as for hypotheses generation.

Disclosures: Duffles: Gilead: Honoraria, Speakers Bureau; Abbvie: Speakers Bureau; AstraZeneca: Speakers Bureau; Takeda: Honoraria, Speakers Bureau; Roche: Speakers Bureau; Johnson & Johnson Innovative Medicine: Honoraria, Speakers Bureau. De Farias: Europharma: Consultancy, Membership on an entity's Board of Directors or advisory committees; Sanofi: Speakers Bureau; MEDAC: Research Funding; Pintfarma: Speakers Bureau; Knight Therapeutics/United Medical: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Kite/Gilead: Consultancy, Membership on an entity's Board of Directors or advisory committees; Celltrion: Research Funding; Viracta: Research Funding; Takeda: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Amgen: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; BMS: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Roche: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Libbs/mAbxcience: Consultancy, Membership on an entity's Board of Directors or advisory committees, Other: Independent data monitoring committee, Speakers Bureau; Celgene: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Pfizer: Speakers Bureau; Zodiac: Speakers Bureau; Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Janssen: Consultancy, Membership on an entity's Board of Directors or advisory committees, Other: Steering committee member, Research Funding, Speakers Bureau; AstraZeneca: Research Funding, Speakers Bureau; Abbvie: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Agios: Research Funding; Sandoz: Research Funding; Onconova: Research Funding; MSD: Research Funding; Lilly: Research Funding; Regeneron: Research Funding; GSK: Research Funding. Dias: Astrazeneca: Research Funding. Teixeira: Astrazeneca: Research Funding. Perini: BMS, Roche, Abbvie, AsstaZeneca, Beigene: Speakers Bureau. Oliver: Abbvie: Consultancy; Servier: Consultancy; Roche: Consultancy. Malpica: Dizal: Research Funding; Eisai: Research Funding.

*signifies non-member of ASH