Adult acute lymphoblastic leukemia in a resource-constrained setting: outcomes after expansion of genetic evaluation
Acute lymphoblastic leukemia (ALL) is a challenging disease with a growing genetic landscape, even though there is substantial gap between developed and non-developed countries when it comes to availability of such new technologies. This manuscript reports a 5-year retrospective cohort of newly diag...
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Taylor & Francis Group
2022-12-01
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| Series: | Hematology |
| Subjects: | |
| Online Access: | https://www.tandfonline.com/doi/10.1080/16078454.2022.2052602 |
| _version_ | 1828289688476057600 |
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| author | Wellington F. Silva Mariane T. Amano Luiza L. Perruso Maria Gabriella Cordeiro Renata Kiyomi Kishimoto Aline de Medeiros Leal Luciana Nardinelli Israel Bendit Elvira DRP Velloso Eduardo M. Rego Vanderson Rocha |
| author_facet | Wellington F. Silva Mariane T. Amano Luiza L. Perruso Maria Gabriella Cordeiro Renata Kiyomi Kishimoto Aline de Medeiros Leal Luciana Nardinelli Israel Bendit Elvira DRP Velloso Eduardo M. Rego Vanderson Rocha |
| author_sort | Wellington F. Silva |
| collection | DOAJ |
| description | Acute lymphoblastic leukemia (ALL) is a challenging disease with a growing genetic landscape, even though there is substantial gap between developed and non-developed countries when it comes to availability of such new technologies. This manuscript reports a 5-year retrospective cohort of newly diagnosed ALL patients and their genetic findings and outcomes. An expanded genetic evaluation by using FISH and RT–PCR was implemented, aiming to identify Ph-like alterations. Patients were treated according to our local protocol, which allocated patients according to age and Philadelphia-chromosome status. A total of 104 patients was included, with median age of 37.5 years. Philadelphia chromosome was detected in 33 cases of B-lineage. Among 45 Ph-negative B-lineage, after excluding KMT2A or TCF3-PBX1 cases, we identified 9 cases with Ph-like fusion. Ph-positive and Ph-like patients had higher initial WBC (p = 0.06). Out of 104 cases, two cases did not start chemotherapy and an early death rate of 10.8% was found. Allogeneic transplantation was performed in 18 cases, being ten performed in first CR. Three-year overall survival (OS) and 3-year event-free survival were 42.8% and 30.8%, respectively. For patients treated with a pediatric regimen, 3-year OS was 52.5%. Extramedullary disease (HR 0.42) and platelet counts (HR 0.9) were independently associated with OS. We still face excessive non-relapse mortality that compromises our results. Alternative strategies implementing FISH and RT–PCR are feasible and able to identify Ph-like fusions. Delays in allogeneic transplantation, as well as the unavailability of new agents, impact long-term survival. Measures to decrease early infection are desirable. |
| first_indexed | 2024-04-13T10:25:00Z |
| format | Article |
| id | doaj.art-52343e2dddc546b8a2fb0e15f9e46b0e |
| institution | Directory Open Access Journal |
| issn | 1607-8454 |
| language | English |
| last_indexed | 2024-04-13T10:25:00Z |
| publishDate | 2022-12-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Hematology |
| spelling | doaj.art-52343e2dddc546b8a2fb0e15f9e46b0e2022-12-22T02:50:21ZengTaylor & Francis GroupHematology1607-84542022-12-0127139640310.1080/16078454.2022.2052602Adult acute lymphoblastic leukemia in a resource-constrained setting: outcomes after expansion of genetic evaluationWellington F. Silva0Mariane T. Amano1Luiza L. Perruso2Maria Gabriella Cordeiro3Renata Kiyomi Kishimoto4Aline de Medeiros Leal5Luciana Nardinelli6Israel Bendit7Elvira DRP Velloso8Eduardo M. Rego9Vanderson Rocha10Division of Hematology, Instituto do Cancer do Estado de São Paulo, São Paulo, BrazilInstituto Sírio-Libanês de Ensino e Pesquisa, Hospital Sírio-Libanês, São Paulo, BrazilDivision of Hematology, Instituto do Cancer do Estado de São Paulo, São Paulo, BrazilDivision of Hematology, Hospital das Clinicas da Faculdade de Medicina (HCFMUSP), Universidade de Sao Paulo, Sao Paulo, BrazilHospital Israelita Albert Einstein, São Paulo, BrazilDivision of Hematology, Hospital das Clinicas da Faculdade de Medicina (HCFMUSP), Universidade de Sao Paulo, Sao Paulo, BrazilDivision of Hematology, Hospital das Clinicas da Faculdade de Medicina (HCFMUSP), Universidade de Sao Paulo, Sao Paulo, BrazilDivision of Hematology, Hospital das Clinicas da Faculdade de Medicina (HCFMUSP), Universidade de Sao Paulo, Sao Paulo, BrazilDivision of Hematology, Instituto do Cancer do Estado de São Paulo, São Paulo, BrazilDivision of Hematology, Instituto do Cancer do Estado de São Paulo, São Paulo, BrazilDivision of Hematology, Instituto do Cancer do Estado de São Paulo, São Paulo, BrazilAcute lymphoblastic leukemia (ALL) is a challenging disease with a growing genetic landscape, even though there is substantial gap between developed and non-developed countries when it comes to availability of such new technologies. This manuscript reports a 5-year retrospective cohort of newly diagnosed ALL patients and their genetic findings and outcomes. An expanded genetic evaluation by using FISH and RT–PCR was implemented, aiming to identify Ph-like alterations. Patients were treated according to our local protocol, which allocated patients according to age and Philadelphia-chromosome status. A total of 104 patients was included, with median age of 37.5 years. Philadelphia chromosome was detected in 33 cases of B-lineage. Among 45 Ph-negative B-lineage, after excluding KMT2A or TCF3-PBX1 cases, we identified 9 cases with Ph-like fusion. Ph-positive and Ph-like patients had higher initial WBC (p = 0.06). Out of 104 cases, two cases did not start chemotherapy and an early death rate of 10.8% was found. Allogeneic transplantation was performed in 18 cases, being ten performed in first CR. Three-year overall survival (OS) and 3-year event-free survival were 42.8% and 30.8%, respectively. For patients treated with a pediatric regimen, 3-year OS was 52.5%. Extramedullary disease (HR 0.42) and platelet counts (HR 0.9) were independently associated with OS. We still face excessive non-relapse mortality that compromises our results. Alternative strategies implementing FISH and RT–PCR are feasible and able to identify Ph-like fusions. Delays in allogeneic transplantation, as well as the unavailability of new agents, impact long-term survival. Measures to decrease early infection are desirable.https://www.tandfonline.com/doi/10.1080/16078454.2022.2052602Acute lymphoblastic leukemiageneticPhiladelphia chromosomePhiladelphia-like leukemiaresponsesurvival |
| spellingShingle | Wellington F. Silva Mariane T. Amano Luiza L. Perruso Maria Gabriella Cordeiro Renata Kiyomi Kishimoto Aline de Medeiros Leal Luciana Nardinelli Israel Bendit Elvira DRP Velloso Eduardo M. Rego Vanderson Rocha Adult acute lymphoblastic leukemia in a resource-constrained setting: outcomes after expansion of genetic evaluation Hematology Acute lymphoblastic leukemia genetic Philadelphia chromosome Philadelphia-like leukemia response survival |
| title | Adult acute lymphoblastic leukemia in a resource-constrained setting: outcomes after expansion of genetic evaluation |
| title_full | Adult acute lymphoblastic leukemia in a resource-constrained setting: outcomes after expansion of genetic evaluation |
| title_fullStr | Adult acute lymphoblastic leukemia in a resource-constrained setting: outcomes after expansion of genetic evaluation |
| title_full_unstemmed | Adult acute lymphoblastic leukemia in a resource-constrained setting: outcomes after expansion of genetic evaluation |
| title_short | Adult acute lymphoblastic leukemia in a resource-constrained setting: outcomes after expansion of genetic evaluation |
| title_sort | adult acute lymphoblastic leukemia in a resource constrained setting outcomes after expansion of genetic evaluation |
| topic | Acute lymphoblastic leukemia genetic Philadelphia chromosome Philadelphia-like leukemia response survival |
| url | https://www.tandfonline.com/doi/10.1080/16078454.2022.2052602 |
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