T-CELL BRAZIL PROJECT: AN EXPLORATORY ANALYSIS OF EXTRANODAL SITES
Objectives: To evaluate number of extranodal (EN) sites in nodal PTCL lymphomas (PTCL-NOS, TFH and ALCL ALK+/ALK-) and its specific location as a surrogate for overall survival (OS) and progression free survival (PFS). Also generate hypothesis for further molecular analysis. Material and methods: T-...
| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2023-10-01
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| Series: | Hematology, Transfusion and Cell Therapy |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2531137923008696 |
| _version_ | 1797654942362632192 |
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| author | T Fischer E Miranda NS Castro J Pereira D Borducchi SS Medina SAB Brasil DFC Farias M Bellesso JV Tavares KZ Cecyn R Schaffel S Nabhan FL Nogueira RLR Baptista FB Duarte RR Sousa MD Pont CC Vilarim CCG Macedo AD Cunh-Junior PPG Radtke E Negreiros N Hamerschlak VLP Figueiredo DV Cle N Zing M Dias RD Gaiolla YBM Gonzaga JTD Sout-Filho EFO Ribeiro GF Silva S Mo G Perini MAL Matedi A Hallac-Neto YS Rabelo M Federico CA Souza CS Chiattone |
| author_facet | T Fischer E Miranda NS Castro J Pereira D Borducchi SS Medina SAB Brasil DFC Farias M Bellesso JV Tavares KZ Cecyn R Schaffel S Nabhan FL Nogueira RLR Baptista FB Duarte RR Sousa MD Pont CC Vilarim CCG Macedo AD Cunh-Junior PPG Radtke E Negreiros N Hamerschlak VLP Figueiredo DV Cle N Zing M Dias RD Gaiolla YBM Gonzaga JTD Sout-Filho EFO Ribeiro GF Silva S Mo G Perini MAL Matedi A Hallac-Neto YS Rabelo M Federico CA Souza CS Chiattone |
| author_sort | T Fischer |
| collection | DOAJ |
| description | Objectives: To evaluate number of extranodal (EN) sites in nodal PTCL lymphomas (PTCL-NOS, TFH and ALCL ALK+/ALK-) and its specific location as a surrogate for overall survival (OS) and progression free survival (PFS). Also generate hypothesis for further molecular analysis. Material and methods: T-cell Brazil project is a national, ambispective study of patients (pts) with histological diagnosis of PTCL diagnosed from January 2015 to December 2022. Approval for the study was obtained at the coordinating center (Samaritano Hospital – São Paulo) and at each participating center. Inclusion criteria was previously untreated patients age ≥ 19 years, with de novo PTCL lymphoma. Clinical information, initial therapy and response, subsequent therapies, along with survival status and cause of death were collected. Treatment outcome was determined by OS and PFS. REDcap Platform (by Vanderbilt) has been used to collect and store data and for analysis the IBM-SPSS v. 24 was applied. Kaplan-Meier method estimated the OS and PFS, whereas Log-Rank tests to compare its curves. This trial is registered at Clinical trials (NCT03207789). Results: Of 621 registered we selected 198 patients (pts) diagnosed with nodal PTCL with at least one EN involvement. Considering all 198 pts, there was a slight male predominance (63%); median age 53 years; 79% were staged III or IV; 81.5 were IPI 2 or more. Most frequently histology was PTCL-NOS (46%), followed by ALCL ALK+ (32%), ALCL ALK- (12.5%) and TFH (9.5%). The majority had B Symptoms (63%); 37.5% had Bone Marrow infiltration. The chemotherapy most frequently chosen were CHOEP (54.5%) followed by CHOP (19.5%); Transplant as consolidation was in 20% of the cases. Almost half of pts achieved complete response after first line (44%), although 38% relapsed. Cohort 1 (E N = 1) and 2 (EN 32) were similar regarding clinical characteristics, except, for stage III-IV (73% vs 96%; p <.0001); IPI 33 (37.5% vs. 82%; p <.0001) and ECOG (22 vs. 48%; p = .001), respectively. Therefore, translating in a more advanced disease in Cohort 2. The most common extranodal location in Cohort 1 was Skin/ Subcutaneous (35%), followed by gastrointestinal tract (18%) and lung (16%). NHL-T subtypes behaved similarly in this EN exploratory analysis, with a better OS and PFS in ALCL ALK+, followed by ALK- and PTCL-NOS. There was no difference in PFS in Cohort 1 and 2, but there was a slight difference in OS (55% vs. 42% in 12 months; p = 0.06), suggesting EN sites involvement assessed by CT and PET-CT as possible surrogate for outcomes in this population. Discussion: PTCL lymphomas account for 10-15% of all NHL. They are a heterogeneous group of infrequent neoplasms with a variable clinical course but prevalently aggressive behavior and high mortality rates. Despite IPI (International Prognostic Index) that include EN site in its variables, lack is known regarding EN location or impact on prognosis. Conclusion: NHL-T is still unmet medical need considering suboptimal outcome in treatment and survival. New biological and clinical finding are still necessary to adequate stratify this group of pts, considering poor performance of IPI and PIT scores. Number and location of EN sites involvement may be a possible surrogate for outcome, which can be a reflect of a distinct biology, that needs further investigation. Registries are of importance considering rarity and poor prognosis of this diseases and an adequate instrument to hypothesis generation. |
| first_indexed | 2024-03-11T17:06:58Z |
| format | Article |
| id | doaj.art-07b0cfa9f254491bbea5b8e5bcc51751 |
| institution | Directory Open Access Journal |
| issn | 2531-1379 |
| language | English |
| last_indexed | 2024-03-11T17:06:58Z |
| publishDate | 2023-10-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Hematology, Transfusion and Cell Therapy |
| spelling | doaj.art-07b0cfa9f254491bbea5b8e5bcc517512023-10-20T06:42:41ZengElsevierHematology, Transfusion and Cell Therapy2531-13792023-10-0145S361S362T-CELL BRAZIL PROJECT: AN EXPLORATORY ANALYSIS OF EXTRANODAL SITEST Fischer0E Miranda1NS Castro2J Pereira3D Borducchi4SS Medina5SAB Brasil6DFC Farias7M Bellesso8JV Tavares9KZ Cecyn10R Schaffel11S Nabhan12FL Nogueira13RLR Baptista14FB Duarte15RR Sousa16MD Pont17CC Vilarim18CCG Macedo19AD Cunh-Junior20PPG Radtke21E Negreiros22N Hamerschlak23VLP Figueiredo24DV Cle25N Zing26M Dias27RD Gaiolla28YBM Gonzaga29JTD Sout-Filho30EFO Ribeiro31GF Silva32S Mo33G Perini34MAL Matedi35A Hallac-Neto36YS Rabelo37M Federico38CA Souza39CS Chiattone40AC Camargo Câncer Center, São Paulo, BrazilCentro de Hematologia e Hemoterapia (Hemocentro), Universidade Estadual de Campinas (UNICAMP), Campinas, BrazilHospital de Câncer de Barretos, Hospital de Amor de Barretos, Barretos, BrazilLaboratory of Medical Investigation in Pathogenesis and Directed Therapy in Onco-Immuno-Hematology, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo, BrazilFaculdade de Medicina do ABC (FMABC), Santo André, BrazilCentro de Hematologia e Hemoterapia (Hemocentro), Universidade Estadual de Campinas (UNICAMP), Campinas, BrazilHematology Department, Faculdade de Ciências Médicas da Santa Casa de São Paulo (FCMSCSP), São Paulo, BrazilA Beneficência Portuguesa de São Paulo (BP), São Paulo, BrazilHemoMed, Instituto de Ensino e Pesquisa (IEP), São Paulo, BrazilHospital Ophir Loyola (HOL), Belém, BrazilHematology Department, Universidade Federal de São Paulo (UNIFESP), São Paulo, BrazilHospital Universitário Clementino Fraga Filho (HUCFF), Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, BrazilUniversidade Federal do Paraná (UFPR), Curitiba, BrazilHospital Luxemburgo (HL), Instituto Mario Penna, Belo Horizonte, BrazilHU, Universidade Estadual do Rio de Janeiro (UERJ) e Instituto D'Or de Pesquisa e Ensino (IDOR), Rio de Janeiro, BrazilHospital Universitário Walter Cantídio (HUWC), Universidade Federal do Ceará (UFC), Fortaleza, BrazilHospital Universitário Walter Cantídio (HUWC), Universidade Federal do Ceará (UFC), Fortaleza, BrazilCentro de Pesquisas Oncológicas de Santa Catarina (CEPON), Florianópolis, BrazilInstituto de Ensino, Pesquisa e Inovação, Liga Contra o Câncer (CECAN), Natal, BrazilInstituto de Ensino, Pesquisa e Inovação, Liga Contra o Câncer (CECAN), Natal, BrazilHematology and Oncology Clinics, Hospital do Câncer de Cascavel, União Oeste Paranaense de Estudos e Combate ao Câncer (UOPECCAN), Cascavel, BrazilCasa de Saúde Santa Marcelina, São Paulo, BrazilHospital de Amor Amazônia, Fundação Pio XII, Porto Velho, BrazilHospital Israelita Albert Einstein (HIAE), São Paulo, BrazilHospital do Servidor Público do Estado de São Paulo (HSPE), Instituto de Assistência Médica ao Servidor Público Estadual (IAMSPE), São Paulo, BrazilFaculdade de Medicina de Ribeirão Preto (FMRP), Universidade de São Paulo (USP), Ribeirão Preto, BrazilPrevent Senior, São Paulo, BrazilHospital Universitário Professor Edgard Santos (HUPES), Universidade Federal da Bahia (UFBA), Salvador, BrazilFaculdade de Medicina de Botucatu, Universidade Estadual Paulista (UNESP), Botucatu, BrazilInstituto Nacional de Câncer (INCA), Rio de Janeiro, RJ, BrasilFaculdade de Medicina de Campos (FMC), Campos dos Goytacazes, BrazilHospital Santa Lúcia, Brasília, BrazilHospital Aldenora Bello, São Luís, BrazilHospital Samaritano, São Paulo, BrazilHospital Paulistano, Oncologia Américas, São Paulo, BrazilSanta Casa de Belo Horizonte, Belo Horizonte, BrazilUniversidade Federal de Juiz de Fora (UFJF), Juiz de Fora, MG, BrasilUniversidade Federal de Goiás (UFG), Goiânia, BrazilUniversity of Modena and Reggio Emília, ItalyCentro de Hematologia e Hemoterapia (Hemocentro), Universidade Estadual de Campinas (UNICAMP), Campinas, BrazilHematology Department, Faculdade de Ciências Médicas da Santa Casa de São Paulo (FCMSCSP), São Paulo, Brazil; Hospital Samaritano, São Paulo, BrazilObjectives: To evaluate number of extranodal (EN) sites in nodal PTCL lymphomas (PTCL-NOS, TFH and ALCL ALK+/ALK-) and its specific location as a surrogate for overall survival (OS) and progression free survival (PFS). Also generate hypothesis for further molecular analysis. Material and methods: T-cell Brazil project is a national, ambispective study of patients (pts) with histological diagnosis of PTCL diagnosed from January 2015 to December 2022. Approval for the study was obtained at the coordinating center (Samaritano Hospital – São Paulo) and at each participating center. Inclusion criteria was previously untreated patients age ≥ 19 years, with de novo PTCL lymphoma. Clinical information, initial therapy and response, subsequent therapies, along with survival status and cause of death were collected. Treatment outcome was determined by OS and PFS. REDcap Platform (by Vanderbilt) has been used to collect and store data and for analysis the IBM-SPSS v. 24 was applied. Kaplan-Meier method estimated the OS and PFS, whereas Log-Rank tests to compare its curves. This trial is registered at Clinical trials (NCT03207789). Results: Of 621 registered we selected 198 patients (pts) diagnosed with nodal PTCL with at least one EN involvement. Considering all 198 pts, there was a slight male predominance (63%); median age 53 years; 79% were staged III or IV; 81.5 were IPI 2 or more. Most frequently histology was PTCL-NOS (46%), followed by ALCL ALK+ (32%), ALCL ALK- (12.5%) and TFH (9.5%). The majority had B Symptoms (63%); 37.5% had Bone Marrow infiltration. The chemotherapy most frequently chosen were CHOEP (54.5%) followed by CHOP (19.5%); Transplant as consolidation was in 20% of the cases. Almost half of pts achieved complete response after first line (44%), although 38% relapsed. Cohort 1 (E N = 1) and 2 (EN 32) were similar regarding clinical characteristics, except, for stage III-IV (73% vs 96%; p <.0001); IPI 33 (37.5% vs. 82%; p <.0001) and ECOG (22 vs. 48%; p = .001), respectively. Therefore, translating in a more advanced disease in Cohort 2. The most common extranodal location in Cohort 1 was Skin/ Subcutaneous (35%), followed by gastrointestinal tract (18%) and lung (16%). NHL-T subtypes behaved similarly in this EN exploratory analysis, with a better OS and PFS in ALCL ALK+, followed by ALK- and PTCL-NOS. There was no difference in PFS in Cohort 1 and 2, but there was a slight difference in OS (55% vs. 42% in 12 months; p = 0.06), suggesting EN sites involvement assessed by CT and PET-CT as possible surrogate for outcomes in this population. Discussion: PTCL lymphomas account for 10-15% of all NHL. They are a heterogeneous group of infrequent neoplasms with a variable clinical course but prevalently aggressive behavior and high mortality rates. Despite IPI (International Prognostic Index) that include EN site in its variables, lack is known regarding EN location or impact on prognosis. Conclusion: NHL-T is still unmet medical need considering suboptimal outcome in treatment and survival. New biological and clinical finding are still necessary to adequate stratify this group of pts, considering poor performance of IPI and PIT scores. Number and location of EN sites involvement may be a possible surrogate for outcome, which can be a reflect of a distinct biology, that needs further investigation. Registries are of importance considering rarity and poor prognosis of this diseases and an adequate instrument to hypothesis generation.http://www.sciencedirect.com/science/article/pii/S2531137923008696 |
| spellingShingle | T Fischer E Miranda NS Castro J Pereira D Borducchi SS Medina SAB Brasil DFC Farias M Bellesso JV Tavares KZ Cecyn R Schaffel S Nabhan FL Nogueira RLR Baptista FB Duarte RR Sousa MD Pont CC Vilarim CCG Macedo AD Cunh-Junior PPG Radtke E Negreiros N Hamerschlak VLP Figueiredo DV Cle N Zing M Dias RD Gaiolla YBM Gonzaga JTD Sout-Filho EFO Ribeiro GF Silva S Mo G Perini MAL Matedi A Hallac-Neto YS Rabelo M Federico CA Souza CS Chiattone T-CELL BRAZIL PROJECT: AN EXPLORATORY ANALYSIS OF EXTRANODAL SITES Hematology, Transfusion and Cell Therapy |
| title | T-CELL BRAZIL PROJECT: AN EXPLORATORY ANALYSIS OF EXTRANODAL SITES |
| title_full | T-CELL BRAZIL PROJECT: AN EXPLORATORY ANALYSIS OF EXTRANODAL SITES |
| title_fullStr | T-CELL BRAZIL PROJECT: AN EXPLORATORY ANALYSIS OF EXTRANODAL SITES |
| title_full_unstemmed | T-CELL BRAZIL PROJECT: AN EXPLORATORY ANALYSIS OF EXTRANODAL SITES |
| title_short | T-CELL BRAZIL PROJECT: AN EXPLORATORY ANALYSIS OF EXTRANODAL SITES |
| title_sort | t cell brazil project an exploratory analysis of extranodal sites |
| url | http://www.sciencedirect.com/science/article/pii/S2531137923008696 |
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