THE NUMBER OF EXTRANODAL SITES IN NODAL PTCL: A PROPOSAL FOR A FEASIBLE PROGNOSTIC MARKER FOR OUTCOMES IN LOW-INCOME COUNTRIES. A COLLABORATIVE STUDY GRUPO DE ESTUDIO LATINO-AMERICANO DE LINFOPROLIFERATIVO (GELL) & T-CELL BRAZIL PROJECT (TCBP)
Introduction: PTCL accounts for 10-15% of all NHL. As previously demonstrated, Latin America has its own epidemiological distribution, with a high frequency of ATLL and ENKT, likely influenced by distinct genetic profiles and viral epidemiology. 3-year OS is about 40%. Treatment advances have also b...
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2024-10-01
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| Series: | Hematology, Transfusion and Cell Therapy |
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| author | T Fischer E Miranda J Pereira G Duffles JV Tavares NS Castro RSA Silva DLC Farias SAB Brasil CCG Macedo C Colaço RLR Baptista KZ Cecyn D Bortucchi GFS Barros S Nabhan PPG Radtke R Schaffel N Zing FL Nogueira AD Cunha-Junior DV Clé JTDS Filho VLP Figueiredo MD Pont R Gaiolla N Hamerschlak EFO Ribeiro A Hallack-Neto MA Dias Y Gonzaga YS Rabelo L Teixeira G Perini MALHM Conhalato P Cury H Idrobo D Castro B Beltran D Enriquez J Vasquez C Roche D Artiles F Valvert L Villela C Oliver L Korin C Pena M Roa MAT Viera AV Gasenapp A Quiroz CS Figari R Rios S Paredes EE Saul C Bermack K Meza B Valcarcel CA Souza L Malpica CS Chiattone |
| author_facet | T Fischer E Miranda J Pereira G Duffles JV Tavares NS Castro RSA Silva DLC Farias SAB Brasil CCG Macedo C Colaço RLR Baptista KZ Cecyn D Bortucchi GFS Barros S Nabhan PPG Radtke R Schaffel N Zing FL Nogueira AD Cunha-Junior DV Clé JTDS Filho VLP Figueiredo MD Pont R Gaiolla N Hamerschlak EFO Ribeiro A Hallack-Neto MA Dias Y Gonzaga YS Rabelo L Teixeira G Perini MALHM Conhalato P Cury H Idrobo D Castro B Beltran D Enriquez J Vasquez C Roche D Artiles F Valvert L Villela C Oliver L Korin C Pena M Roa MAT Viera AV Gasenapp A Quiroz CS Figari R Rios S Paredes EE Saul C Bermack K Meza B Valcarcel CA Souza L Malpica CS Chiattone |
| author_sort | T Fischer |
| collection | DOAJ |
| description | Introduction: PTCL accounts for 10-15% of all NHL. As previously demonstrated, Latin America has its own epidemiological distribution, with a high frequency of ATLL and ENKT, likely influenced by distinct genetic profiles and viral epidemiology. 3-year OS is about 40%. Treatment advances have also been limited, except for BV-CHP in some countries. The IPI, which includes extranodal (EN) site as a variable, has been validated for PTCL. However, the specific impact of EN involvement on nodal PTCL (such as PTCL-NOS, AIT, ALCL ALK+/ALK-) and its biological implications remain unclear. Simplification could improve the reproducibility and applicability of these models, especially in low-income countries. Objective: To evaluate number of EN sites in nodal PTCL lymphomas as a risk factors or surrogate for outcomes, as OS and PFS in Latin America cohort. Methodology: Patients (pts) aged ≥18 years with newly diagnosed nodal PTCL-NOS, AITL and ALCL ALK+/ALK-) from GELL (n = 339, 2000-2023, retrospective), TCBP (n = 427, 2015-2022, ambispective). Treatment outcome was determined by OS and PFS. REDcap Platform (by Vanderbilt) was used to collect and store data, whereas statistical analysis the IBM-SPSS v.24. This trial is registered at Clinical trials (NCT03207789). Results: 766 pts [427pts - TCBP and 339 - GELL] diagnosed with nodal PTCL were grouped according to the number of EN: no EN involvement (No EN - 383); one EN involvement (EN1 -168); and 32 (EN2 - 215). Considering all, 61% male; median age 56 y/o; 74% were staged III/IV; 69% IPI 32; 60% was PTCL-NOS, 19% ALCL ALK- and 12% AITL. 61% had B symptoms and 55% elevated LDH. CHOEP was used in 47% and 34% CHOP, and 47% achieved CR after first line; 16% used transplant as consolidation. No EN, EN1 and EN2 were similar regarding clinical characteristics, except, for stage III/IV (58% vs. 79% vs 96%; p < .0001); IPI 32 (58% vs. 59% vs. 99%; p < .0001); ECOG>1 (58% vs. 92% vs. 99%; p < .0001); BMO involvement (16% vs. 24% vs. 63%%; p <.0001), elevated LDH (54% vs. 47% vs. 61%; p =.03) and Hypoalbuminemia (33% vs. 38% vs. 58%; p <.0001). NHL-T subtypes showed better OS and ALCL ALK+ better PFS. Among No EN, EN1 and EN2 the response CR after 1st line had a statistically difference (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). Conclusion: EN2 presented more advanced disease, with possible distinct biology, hence, the number of EN involvements can be useful as a practical and informative surrogates for outcomes, suggesting number of EN sites involvement assessed by CT and PET-CT as feasible clinical surrogate for outcomes in this population. New biomarkers are essential to better stratify patients with PTCL. Still, in practice, it is also necessary to have ways to stratify these patients using clinical data, mainly in regions with low-income resources. These indicators reflect distinct biological characteristics 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 generate hypotheses. |
| format | Article |
| id | doaj-art-5f15d08093f549d3a333ac68a7b006be |
| institution | OA Journals |
| issn | 2531-1379 |
| language | English |
| publishDate | 2024-10-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Hematology, Transfusion and Cell Therapy |
| spelling | doaj-art-5f15d08093f549d3a333ac68a7b006be2025-08-20T02:17:40ZengElsevierHematology, Transfusion and Cell Therapy2531-13792024-10-0146S256S25810.1016/j.htct.2024.09.430THE NUMBER OF EXTRANODAL SITES IN NODAL PTCL: A PROPOSAL FOR A FEASIBLE PROGNOSTIC MARKER FOR OUTCOMES IN LOW-INCOME COUNTRIES. A COLLABORATIVE STUDY GRUPO DE ESTUDIO LATINO-AMERICANO DE LINFOPROLIFERATIVO (GELL) & T-CELL BRAZIL PROJECT (TCBP)T Fischer0E Miranda1J Pereira2G Duffles3JV Tavares4NS Castro5RSA Silva6DLC Farias7SAB Brasil8CCG Macedo9C Colaço10RLR Baptista11KZ Cecyn12D Bortucchi13GFS Barros14S Nabhan15PPG Radtke16R Schaffel17N Zing18FL Nogueira19AD Cunha-Junior20DV Clé21JTDS Filho22VLP Figueiredo23MD Pont24R Gaiolla25N Hamerschlak26EFO Ribeiro27A Hallack-Neto28MA Dias29Y Gonzaga30YS Rabelo31L Teixeira32G Perini33MALHM Conhalato34P Cury35H Idrobo36D Castro37B Beltran38D Enriquez39J Vasquez40C Roche41D Artiles42F Valvert43L Villela44C Oliver45L Korin46C Pena47M Roa48MAT Viera49AV Gasenapp50A Quiroz51CS Figari52R Rios53S Paredes54EE Saul55C Bermack56K Meza57B Valcarcel58CA Souza59L Malpica60CS Chiattone61AC Camargo Câncer Center, São Paulo, BrazilUniversidade Estadual de Campinas (UNICAMP), Campinas, BrazilUniversidade de São Paulo (USP), São Paulo, BrazilUniversidade Estadual de Campinas (UNICAMP), Campinas, BrazilHospital Ophir Loyola (HOL), Belém, BrazilHospital de Amor de Barretos, Barretos, BrazilHemoMed, Instituto de Ensino e Pesquisa (IEP), São Paulo, BrazilA Beneficência Portuguesa de São Paulo (BP), São Paulo, BrazilIrmandade da Santa Casa de Misericórdia de São Paulo (ISCMSP), São Paulo, BrazilInstituto de Ensino, Pesquisa e Inovação, Liga Contra o Câncer (CECAN), Natal, BrazilInstituto de Ensino, Pesquisa e Inovação, Liga Contra o Câncer (CECAN), Natal, BrazilUniversidade do Estado do Rio de Janeiro (UERJ) & Oncologia D'Or, Rio de Janeiro, BrazilUniversidade Federal de São Paulo (UNIFESP), São Paulo, BrazilFaculdade de Medicina do ABC (FMABC), Santo André, BrazilHospital Aldenora Bello, São Luís, BrazilUniversidade Federal do Paraná (UFPR), Curitiba, BrazilHospital Santa Marcelina, São Paulo, BrazilUniversidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, BrazilPrevent Senior de São Paulo, São Paulo, BrazilHospital Luxemburgo (HL), Belo Horizonte, BrazilHospital do Câncer de Cascavel, Cascavel, BrazilUniversidade de São Paulo (USP), Ribeirão Preto, BrazilFaculdade de Medicina de Campos (FMC), Campos dos Goytacazes, BrazilHospital do Servidor Público do Estado de São Paulo (HSPE), Instituto de Assistência Médica ao Servidor Público Estadual (IAMSPE), São Paulo, BrazilCentro de Pesquisas Oncológicas de Santa Catarina (CEPON), Florianópolis, BrazilUniversidade Estadual Paulista Júlio de Mesquita Filho (UNESP), Botucatu, BrazilHospital Israelita Albert Einstein (HIAE), São Paulo, BrazilGrupo Santa Lúcia, Brasília, BrazilUniversidade Federal de Juiz de Fora (UFJF), Juiz de Fora, BrazilUniversidade Federal da Bahia (UFBA), Salvador, BrazilInstituto Nacional de Câncer (INCA), Rio de Janeiro, BrazilUniversidade Federal de Goiás (UFG), Goiânia, BrazilHospital Municipal Vila Santa Catarina, São Paulo, BrazilHospital Paulistano, Oncologia Américas, São Paulo, BrazilSanta Casa de Belo Horizonte, Belo Horizonte, BrazilClínica São Germano, São Paulo, BrazilUniversidad del Valle del Cauca, Cali, ColombiaHospital Edgardo Rebagliati Martins, Lima, PeruHospital Edgardo Rebagliati Martins, Lima, PeruInstituto Nacional de Enfermedades Neoplasicas, Lima, PeruInstituto Nacional de Enfermedades Neoplasicas, Lima, PeruHospital Arnaldo Milián Castro, Villa Clara, CubaHospital Arnaldo Milián Castro, Villa Clara, CubaINCAN, Ciudad de Mexico, MexicoINCAN, Ciudad de Mexico, MexicoHospital Britanico de Montevideo, Montevideo, UruguaiCABA - Alexander Fleming Institute, Olivos, ArgentinaHospital Del Salvador, Santiago, ChileHospital Del Salvador, Santiago, ChileClínica Santa Sofia, Caracas, VenezuelaHospital Central Instituto de Previsión Social, Asunción, ParaguaiHospital Central Instituto de Previsión Social, Asunción, ParaguaiOncosalud, AUNA, Lima, PeruHospital Clinico Quirúrgico Hermanos Amejeiras, La Habana, CubaHospital Edgardo Rebagliati Martins, Lima, PeruUniversity of Texas, MD Anderson Cancer Center, Houston, United StatesUniversity of Texas, MD Anderson Cancer Center, Houston, United StatesBaylor College of Medicine, Houston, United StatesUniversity of George Washington, Washington DC, United StatesUniversidade Estadual de Campinas (UNICAMP), Campinas, BrazilHospital Clinico Quirúrgico Hermanos Amejeiras, La Habana, CubaIrmandade da Santa Casa de Misericórdia de São Paulo (ISCMSP), São Paulo, Brazil; Hospital Samaritano, São Paulo, SP, BrazilIntroduction: PTCL accounts for 10-15% of all NHL. As previously demonstrated, Latin America has its own epidemiological distribution, with a high frequency of ATLL and ENKT, likely influenced by distinct genetic profiles and viral epidemiology. 3-year OS is about 40%. Treatment advances have also been limited, except for BV-CHP in some countries. The IPI, which includes extranodal (EN) site as a variable, has been validated for PTCL. However, the specific impact of EN involvement on nodal PTCL (such as PTCL-NOS, AIT, ALCL ALK+/ALK-) and its biological implications remain unclear. Simplification could improve the reproducibility and applicability of these models, especially in low-income countries. Objective: To evaluate number of EN sites in nodal PTCL lymphomas as a risk factors or surrogate for outcomes, as OS and PFS in Latin America cohort. Methodology: Patients (pts) aged ≥18 years with newly diagnosed nodal PTCL-NOS, AITL and ALCL ALK+/ALK-) from GELL (n = 339, 2000-2023, retrospective), TCBP (n = 427, 2015-2022, ambispective). Treatment outcome was determined by OS and PFS. REDcap Platform (by Vanderbilt) was used to collect and store data, whereas statistical analysis the IBM-SPSS v.24. This trial is registered at Clinical trials (NCT03207789). Results: 766 pts [427pts - TCBP and 339 - GELL] diagnosed with nodal PTCL were grouped according to the number of EN: no EN involvement (No EN - 383); one EN involvement (EN1 -168); and 32 (EN2 - 215). Considering all, 61% male; median age 56 y/o; 74% were staged III/IV; 69% IPI 32; 60% was PTCL-NOS, 19% ALCL ALK- and 12% AITL. 61% had B symptoms and 55% elevated LDH. CHOEP was used in 47% and 34% CHOP, and 47% achieved CR after first line; 16% used transplant as consolidation. No EN, EN1 and EN2 were similar regarding clinical characteristics, except, for stage III/IV (58% vs. 79% vs 96%; p < .0001); IPI 32 (58% vs. 59% vs. 99%; p < .0001); ECOG>1 (58% vs. 92% vs. 99%; p < .0001); BMO involvement (16% vs. 24% vs. 63%%; p <.0001), elevated LDH (54% vs. 47% vs. 61%; p =.03) and Hypoalbuminemia (33% vs. 38% vs. 58%; p <.0001). NHL-T subtypes showed better OS and ALCL ALK+ better PFS. Among No EN, EN1 and EN2 the response CR after 1st line had a statistically difference (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). Conclusion: EN2 presented more advanced disease, with possible distinct biology, hence, the number of EN involvements can be useful as a practical and informative surrogates for outcomes, suggesting number of EN sites involvement assessed by CT and PET-CT as feasible clinical surrogate for outcomes in this population. New biomarkers are essential to better stratify patients with PTCL. Still, in practice, it is also necessary to have ways to stratify these patients using clinical data, mainly in regions with low-income resources. These indicators reflect distinct biological characteristics 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 generate hypotheses.http://www.sciencedirect.com/science/article/pii/S2531137924007636 |
| spellingShingle | T Fischer E Miranda J Pereira G Duffles JV Tavares NS Castro RSA Silva DLC Farias SAB Brasil CCG Macedo C Colaço RLR Baptista KZ Cecyn D Bortucchi GFS Barros S Nabhan PPG Radtke R Schaffel N Zing FL Nogueira AD Cunha-Junior DV Clé JTDS Filho VLP Figueiredo MD Pont R Gaiolla N Hamerschlak EFO Ribeiro A Hallack-Neto MA Dias Y Gonzaga YS Rabelo L Teixeira G Perini MALHM Conhalato P Cury H Idrobo D Castro B Beltran D Enriquez J Vasquez C Roche D Artiles F Valvert L Villela C Oliver L Korin C Pena M Roa MAT Viera AV Gasenapp A Quiroz CS Figari R Rios S Paredes EE Saul C Bermack K Meza B Valcarcel CA Souza L Malpica CS Chiattone THE NUMBER OF EXTRANODAL SITES IN NODAL PTCL: A PROPOSAL FOR A FEASIBLE PROGNOSTIC MARKER FOR OUTCOMES IN LOW-INCOME COUNTRIES. A COLLABORATIVE STUDY GRUPO DE ESTUDIO LATINO-AMERICANO DE LINFOPROLIFERATIVO (GELL) & T-CELL BRAZIL PROJECT (TCBP) Hematology, Transfusion and Cell Therapy |
| title | THE NUMBER OF EXTRANODAL SITES IN NODAL PTCL: A PROPOSAL FOR A FEASIBLE PROGNOSTIC MARKER FOR OUTCOMES IN LOW-INCOME COUNTRIES. A COLLABORATIVE STUDY GRUPO DE ESTUDIO LATINO-AMERICANO DE LINFOPROLIFERATIVO (GELL) & T-CELL BRAZIL PROJECT (TCBP) |
| title_full | THE NUMBER OF EXTRANODAL SITES IN NODAL PTCL: A PROPOSAL FOR A FEASIBLE PROGNOSTIC MARKER FOR OUTCOMES IN LOW-INCOME COUNTRIES. A COLLABORATIVE STUDY GRUPO DE ESTUDIO LATINO-AMERICANO DE LINFOPROLIFERATIVO (GELL) & T-CELL BRAZIL PROJECT (TCBP) |
| title_fullStr | THE NUMBER OF EXTRANODAL SITES IN NODAL PTCL: A PROPOSAL FOR A FEASIBLE PROGNOSTIC MARKER FOR OUTCOMES IN LOW-INCOME COUNTRIES. A COLLABORATIVE STUDY GRUPO DE ESTUDIO LATINO-AMERICANO DE LINFOPROLIFERATIVO (GELL) & T-CELL BRAZIL PROJECT (TCBP) |
| title_full_unstemmed | THE NUMBER OF EXTRANODAL SITES IN NODAL PTCL: A PROPOSAL FOR A FEASIBLE PROGNOSTIC MARKER FOR OUTCOMES IN LOW-INCOME COUNTRIES. A COLLABORATIVE STUDY GRUPO DE ESTUDIO LATINO-AMERICANO DE LINFOPROLIFERATIVO (GELL) & T-CELL BRAZIL PROJECT (TCBP) |
| title_short | THE NUMBER OF EXTRANODAL SITES IN NODAL PTCL: A PROPOSAL FOR A FEASIBLE PROGNOSTIC MARKER FOR OUTCOMES IN LOW-INCOME COUNTRIES. A COLLABORATIVE STUDY GRUPO DE ESTUDIO LATINO-AMERICANO DE LINFOPROLIFERATIVO (GELL) & T-CELL BRAZIL PROJECT (TCBP) |
| title_sort | number of extranodal sites in nodal ptcl a proposal for a feasible prognostic marker for outcomes in low income countries a collaborative study grupo de estudio latino americano de linfoproliferativo gell amp t cell brazil project tcbp |
| url | http://www.sciencedirect.com/science/article/pii/S2531137924007636 |
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