Caracterização clínica e epidemiológica dos pacientes com diagnóstico de Leucemia Mieloide Aguda no Estado do Amazonas tratado no HEMOAM

Detalhes bibliográficos
Autor(a) principal: Saint Pierre , Stephane
Data de Publicação: 2019
Tipo de documento: Dissertação
Idioma: por
Título da fonte: Repositório Institucional da Universidade do Estado do Amazonas (UEA)
Texto Completo: http://repositorioinstitucional.uea.edu.br//handle/riuea/2131
Resumo: ABSTRACT Acute myeloid leukemia (AML) is a neoplasm of progenitor hematopoietic cells that results in loss of the ability to differentiate from the myeloid lineage, leading to insufficient generation of mature blood cells. LMA is a genetically heterogeneous disease, which occurs most commonly in adults, with increasing incidence with increasing age. The diagnosis, according to the World Health Organization - WHO, requires the presence of 20% of blasts in the bone marrow. It is a highly aggressive disease with a still very low survival rate despite chemotherapeutic treatment, with the exception of acute promyelocytic leukemia. The objective of this study is to characterize the clinical and epidemiological profile of AML patients in the State of Amazonas treated in HEMOAM. The study was retrospective, cross- sectional, including all patients diagnosed with AML between January 2013 and December 2017.were 194 patients diagnosed in this period, patients who were not treated exclusively in HEMOAM, and the medical records were not found were excluded. The median age of the population was 46 years, 70,94% were of the non-white race, the distribution between men and women 96:83.The most frequent clinical manifestations were asthenia (65.89%), other associated symptoms (50,28%), fever (48,55%), and bleeding (31,21%). The majority of the population (45.97%) had a performance status according to the ECOG score of (2). The most frequent FAB subtypes found in this work was M5 followed by M3.The median of hemoglobin was 8.4 (4.1 -14) g\/dl, with median VCM 89 (69.10 interval -115.8) fl. The median of the Leukocyte count was 11660\/mm3 and platelet median was 43000\/mm3. Median (%) peripheral blood blasts was 26% and the bone marrow 71%. With respect to the risk cytogenetic (68%) karyotypes are intermediate risk. In immunophenotyping was the most common marker CD13 being expressed (91,51%) of the cases. Of the findings 53,93% expressed aberrant antigens, with CD7 being most expressed in the T lymphoid line and CD19 in the B lymphoid line. A total of 5 patients were classified as acute bifenotypic leukemia (LAB). The most used treatment lineage in the adult was 3+7, in the children was the BFM 2004. A total of 134 patients were submitted to intensive therapy of which 74 entered in remission. Nine (9) of the patients underwent allogeneic hematopoietic stem cell transplantation. The rate of relapse and refractory were raised. The overall survival at 1 year was 31.28%. The death rate was 67.59%. The survival curves showed that better prognosis was related to the age below of 60 years (median 496dias; p-value < 0.0001; OR 0.2774), patient with AML de novo (median 264dias; p-value 0.0103; OR 0.3858).There was no statistically significant difference when comparing overall survival curves with the xii cytogenetics, the leukocytes more or less de100x10e3\/μ l, and patients who did or didn't BMT. Conclusion, the results obtained made it possible to obtain a profile of demographic and clinical features of AML in the Amazon, provide important tools for a possible development of surveillance systems. Keywords. Acute myeloid leukemia, haematological malignancies
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spelling Caracterização clínica e epidemiológica dos pacientes com diagnóstico de Leucemia Mieloide Aguda no Estado do Amazonas tratado no HEMOAMLeucemia Mileoide agudaneoplasias hematológicasHematologiaABSTRACT Acute myeloid leukemia (AML) is a neoplasm of progenitor hematopoietic cells that results in loss of the ability to differentiate from the myeloid lineage, leading to insufficient generation of mature blood cells. LMA is a genetically heterogeneous disease, which occurs most commonly in adults, with increasing incidence with increasing age. The diagnosis, according to the World Health Organization - WHO, requires the presence of 20% of blasts in the bone marrow. It is a highly aggressive disease with a still very low survival rate despite chemotherapeutic treatment, with the exception of acute promyelocytic leukemia. The objective of this study is to characterize the clinical and epidemiological profile of AML patients in the State of Amazonas treated in HEMOAM. The study was retrospective, cross- sectional, including all patients diagnosed with AML between January 2013 and December 2017.were 194 patients diagnosed in this period, patients who were not treated exclusively in HEMOAM, and the medical records were not found were excluded. The median age of the population was 46 years, 70,94% were of the non-white race, the distribution between men and women 96:83.The most frequent clinical manifestations were asthenia (65.89%), other associated symptoms (50,28%), fever (48,55%), and bleeding (31,21%). The majority of the population (45.97%) had a performance status according to the ECOG score of (2). The most frequent FAB subtypes found in this work was M5 followed by M3.The median of hemoglobin was 8.4 (4.1 -14) g\/dl, with median VCM 89 (69.10 interval -115.8) fl. The median of the Leukocyte count was 11660\/mm3 and platelet median was 43000\/mm3. Median (%) peripheral blood blasts was 26% and the bone marrow 71%. With respect to the risk cytogenetic (68%) karyotypes are intermediate risk. In immunophenotyping was the most common marker CD13 being expressed (91,51%) of the cases. Of the findings 53,93% expressed aberrant antigens, with CD7 being most expressed in the T lymphoid line and CD19 in the B lymphoid line. A total of 5 patients were classified as acute bifenotypic leukemia (LAB). The most used treatment lineage in the adult was 3+7, in the children was the BFM 2004. A total of 134 patients were submitted to intensive therapy of which 74 entered in remission. Nine (9) of the patients underwent allogeneic hematopoietic stem cell transplantation. The rate of relapse and refractory were raised. The overall survival at 1 year was 31.28%. The death rate was 67.59%. The survival curves showed that better prognosis was related to the age below of 60 years (median 496dias; p-value < 0.0001; OR 0.2774), patient with AML de novo (median 264dias; p-value 0.0103; OR 0.3858).There was no statistically significant difference when comparing overall survival curves with the xii cytogenetics, the leukocytes more or less de100x10e3\/μ l, and patients who did or didn't BMT. Conclusion, the results obtained made it possible to obtain a profile of demographic and clinical features of AML in the Amazon, provide important tools for a possible development of surveillance systems. Keywords. Acute myeloid leukemia, haematological malignanciesRESUMO A Leucemia Mieloide Aguda (LMA) é uma neoplasia de células hematopoiéticas progenitoras que resulta na perda da capacidade de diferenciação da linhagem mieloide, e leva à geração insuficiente de células sanguíneas maduras. A LMA é uma doença geneticamente heterogênea, que ocorre mais comumente em adultos, com incidência crescente com o aumento da idade. O diagnóstico, segundo a Organização Mundial de Saúde – OMS, requer a presença de 20 % de blastos na medula óssea o no sangue periférico. É uma doença altamente agressiva com uma taxa de sobrevida ainda muito baixa apesar do tratamento quimioterápica, com exceção da leucemia promielocítica aguda. O objetivo deste estudo é a caracterização do perfil clínico e epidemiológico de pacientes com LMA no Estado do Amazonas tratados no HEMOAM. O estudo foi retrospectivo, tipo corte transversal, incluindo todos os pacientes diagnosticados com LMA entre Janeiro de 2013 até Dezembro de 2017. Foram 194 pacientes diagnosticados neste período, pacientes que não foram tratados exclusivamente no HEMOAM, e os prontuários não encontrados foram excluídos. A idade média da população foi de 46 anos, 70,94% eram da raça não branca, a distribuição entre homens e mulheres foi 96:83. Quanto à apresentação clínica ao diagnóstico, as manifestações clínicas mais frequentes foram astenia (65,89%), outros sintomas associados (50,28%), febre (48,55%),e sangramento(31,21%).A maioria da população de estudo (45,97%) tinha uma performance status segundo a classificação ECOG de (2).Os subtipos FAB mais frequente encontrados neste trabalho foi M5 seguido de M3.A mediana da hemoglobina foi 8,4 (intervalo 4,1- 14)g/dl, com mediana do VCM 89 (intervalo 69,10-115,8)fl. A mediana da contagem de leucócitos foi 11660/mm3 e mediana da plaqueta foi 43000/mm3. Mediana (%) blastos sangue periférico foi 26% e da medula óssea 71%. Com respeito ao risco citogenético, (68%) dos cariótipos encontrados são de risco intermediário. Na inmunofenotipagem CD13 foi o marcador mais frequente sendo expresso (91,51%) dos casos. Dos achados 53,93% expressavam antígenos aberrantes, sendo o CD7 mais expressado na linhagem linfóide T e CD19 na linhagem linfóide B. Um total de 5 pacientes foram classificados como Leucemia Aguda Bifenotipica (LAB). A linhagem de tratamento mais usado no adulto foi 3+7, nas crianças foi o BFM 2004. Um total de 134 pacientes foram submetidos a terapia intensiva, dos quais 74 entraram em remissão. Nove (9) dos pacientes foram submetidos ao transplante alogênico de células-tronco hematopoiéticas. As taxas de recaída e refratariedade foram elevadas. A sobrevida global em 1 ano foi de 31,28%. A taxa de óbito foi 67,59%. As curvas de sobrevida mostraram que o melhor prognóstico foi relacionado a idade abaixo de 60 x anos(mediana 496dias; p-valor<0,0001 ; OR 0,2774),paciente com LMA de novo(mediana 264dias; p-valor 0,0103; OR 0,3858).Não houve diferença estatisticamente significante quando comparadas as curvas de sobrevida global com a citogenética, a leucometria maior ou menor de100x10e3/μl, e pacientes que fez ou não TMO. Em conclusão, os resultados obtidos permitiram descrever um perfil das características demográficas e clínicas da LMA no Amazonas, fornecendo ferramentas importantes para um eventual desenvolvimento de sistemas de vigilância. Palavras Chaves. Leucemia mieloide aguda, neoplasias hematológicas.Universidade do Estado do AmazonasBrasilPrograma de Pós-Graduação em Ciências Aplicadas à HematologiaUEADhyani , AnamikaMarie, Adriana Malheiro AlleCosta, Allyson Guimarães dahttp://lattes.cnpq.br/7531662673281014Tarragô, Andréa Monteirohttp://lattes.cnpq.br/4644326589690231Barros, Francisco Erivaldo Vidalhttp://lattes.cnpq.br/3722691640950389Saint Pierre , Stephane2020-03-092020-03-09T15:07:37Z2019-04-222020-03-09T15:07:37Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/masterThesisapplication/pdfhttp://repositorioinstitucional.uea.edu.br//handle/riuea/2131porREFERÊNCIAS BIBLIOGRÁFICAS 1. Lima MC De, Bousfield D, Paula A, Freund F, Dacoregio JS, El T, et al. Acute Myeloid Leukemia : analysis of epidemiological profile and survival rate ଝ. J Pediatr (Rio J) [Internet]. 2016;92(3):283–9. Available from: http://dx.doi.org/10.1016/j.jped.2015.08.008 2. Kansal R. 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dc.title.none.fl_str_mv Caracterização clínica e epidemiológica dos pacientes com diagnóstico de Leucemia Mieloide Aguda no Estado do Amazonas tratado no HEMOAM
title Caracterização clínica e epidemiológica dos pacientes com diagnóstico de Leucemia Mieloide Aguda no Estado do Amazonas tratado no HEMOAM
spellingShingle Caracterização clínica e epidemiológica dos pacientes com diagnóstico de Leucemia Mieloide Aguda no Estado do Amazonas tratado no HEMOAM
Saint Pierre , Stephane
Leucemia Mileoide aguda
neoplasias hematológicas
Hematologia
title_short Caracterização clínica e epidemiológica dos pacientes com diagnóstico de Leucemia Mieloide Aguda no Estado do Amazonas tratado no HEMOAM
title_full Caracterização clínica e epidemiológica dos pacientes com diagnóstico de Leucemia Mieloide Aguda no Estado do Amazonas tratado no HEMOAM
title_fullStr Caracterização clínica e epidemiológica dos pacientes com diagnóstico de Leucemia Mieloide Aguda no Estado do Amazonas tratado no HEMOAM
title_full_unstemmed Caracterização clínica e epidemiológica dos pacientes com diagnóstico de Leucemia Mieloide Aguda no Estado do Amazonas tratado no HEMOAM
title_sort Caracterização clínica e epidemiológica dos pacientes com diagnóstico de Leucemia Mieloide Aguda no Estado do Amazonas tratado no HEMOAM
author Saint Pierre , Stephane
author_facet Saint Pierre , Stephane
author_role author
dc.contributor.none.fl_str_mv Dhyani , Anamika
Marie, Adriana Malheiro Alle
Costa, Allyson Guimarães da
http://lattes.cnpq.br/7531662673281014
Tarragô, Andréa Monteiro
http://lattes.cnpq.br/4644326589690231
Barros, Francisco Erivaldo Vidal
http://lattes.cnpq.br/3722691640950389
dc.contributor.author.fl_str_mv Saint Pierre , Stephane
dc.subject.por.fl_str_mv Leucemia Mileoide aguda
neoplasias hematológicas
Hematologia
topic Leucemia Mileoide aguda
neoplasias hematológicas
Hematologia
dc.description.none.fl_txt_mv ABSTRACT Acute myeloid leukemia (AML) is a neoplasm of progenitor hematopoietic cells that results in loss of the ability to differentiate from the myeloid lineage, leading to insufficient generation of mature blood cells. LMA is a genetically heterogeneous disease, which occurs most commonly in adults, with increasing incidence with increasing age. The diagnosis, according to the World Health Organization - WHO, requires the presence of 20% of blasts in the bone marrow. It is a highly aggressive disease with a still very low survival rate despite chemotherapeutic treatment, with the exception of acute promyelocytic leukemia. The objective of this study is to characterize the clinical and epidemiological profile of AML patients in the State of Amazonas treated in HEMOAM. The study was retrospective, cross- sectional, including all patients diagnosed with AML between January 2013 and December 2017.were 194 patients diagnosed in this period, patients who were not treated exclusively in HEMOAM, and the medical records were not found were excluded. The median age of the population was 46 years, 70,94% were of the non-white race, the distribution between men and women 96:83.The most frequent clinical manifestations were asthenia (65.89%), other associated symptoms (50,28%), fever (48,55%), and bleeding (31,21%). The majority of the population (45.97%) had a performance status according to the ECOG score of (2). The most frequent FAB subtypes found in this work was M5 followed by M3.The median of hemoglobin was 8.4 (4.1 -14) g\/dl, with median VCM 89 (69.10 interval -115.8) fl. The median of the Leukocyte count was 11660\/mm3 and platelet median was 43000\/mm3. Median (%) peripheral blood blasts was 26% and the bone marrow 71%. With respect to the risk cytogenetic (68%) karyotypes are intermediate risk. In immunophenotyping was the most common marker CD13 being expressed (91,51%) of the cases. Of the findings 53,93% expressed aberrant antigens, with CD7 being most expressed in the T lymphoid line and CD19 in the B lymphoid line. A total of 5 patients were classified as acute bifenotypic leukemia (LAB). The most used treatment lineage in the adult was 3+7, in the children was the BFM 2004. A total of 134 patients were submitted to intensive therapy of which 74 entered in remission. Nine (9) of the patients underwent allogeneic hematopoietic stem cell transplantation. The rate of relapse and refractory were raised. The overall survival at 1 year was 31.28%. The death rate was 67.59%. The survival curves showed that better prognosis was related to the age below of 60 years (median 496dias; p-value < 0.0001; OR 0.2774), patient with AML de novo (median 264dias; p-value 0.0103; OR 0.3858).There was no statistically significant difference when comparing overall survival curves with the xii cytogenetics, the leukocytes more or less de100x10e3\/μ l, and patients who did or didn't BMT. Conclusion, the results obtained made it possible to obtain a profile of demographic and clinical features of AML in the Amazon, provide important tools for a possible development of surveillance systems. Keywords. Acute myeloid leukemia, haematological malignancies
RESUMO A Leucemia Mieloide Aguda (LMA) é uma neoplasia de células hematopoiéticas progenitoras que resulta na perda da capacidade de diferenciação da linhagem mieloide, e leva à geração insuficiente de células sanguíneas maduras. A LMA é uma doença geneticamente heterogênea, que ocorre mais comumente em adultos, com incidência crescente com o aumento da idade. O diagnóstico, segundo a Organização Mundial de Saúde – OMS, requer a presença de 20 % de blastos na medula óssea o no sangue periférico. É uma doença altamente agressiva com uma taxa de sobrevida ainda muito baixa apesar do tratamento quimioterápica, com exceção da leucemia promielocítica aguda. O objetivo deste estudo é a caracterização do perfil clínico e epidemiológico de pacientes com LMA no Estado do Amazonas tratados no HEMOAM. O estudo foi retrospectivo, tipo corte transversal, incluindo todos os pacientes diagnosticados com LMA entre Janeiro de 2013 até Dezembro de 2017. Foram 194 pacientes diagnosticados neste período, pacientes que não foram tratados exclusivamente no HEMOAM, e os prontuários não encontrados foram excluídos. A idade média da população foi de 46 anos, 70,94% eram da raça não branca, a distribuição entre homens e mulheres foi 96:83. Quanto à apresentação clínica ao diagnóstico, as manifestações clínicas mais frequentes foram astenia (65,89%), outros sintomas associados (50,28%), febre (48,55%),e sangramento(31,21%).A maioria da população de estudo (45,97%) tinha uma performance status segundo a classificação ECOG de (2).Os subtipos FAB mais frequente encontrados neste trabalho foi M5 seguido de M3.A mediana da hemoglobina foi 8,4 (intervalo 4,1- 14)g/dl, com mediana do VCM 89 (intervalo 69,10-115,8)fl. A mediana da contagem de leucócitos foi 11660/mm3 e mediana da plaqueta foi 43000/mm3. Mediana (%) blastos sangue periférico foi 26% e da medula óssea 71%. Com respeito ao risco citogenético, (68%) dos cariótipos encontrados são de risco intermediário. Na inmunofenotipagem CD13 foi o marcador mais frequente sendo expresso (91,51%) dos casos. Dos achados 53,93% expressavam antígenos aberrantes, sendo o CD7 mais expressado na linhagem linfóide T e CD19 na linhagem linfóide B. Um total de 5 pacientes foram classificados como Leucemia Aguda Bifenotipica (LAB). A linhagem de tratamento mais usado no adulto foi 3+7, nas crianças foi o BFM 2004. Um total de 134 pacientes foram submetidos a terapia intensiva, dos quais 74 entraram em remissão. Nove (9) dos pacientes foram submetidos ao transplante alogênico de células-tronco hematopoiéticas. As taxas de recaída e refratariedade foram elevadas. A sobrevida global em 1 ano foi de 31,28%. A taxa de óbito foi 67,59%. As curvas de sobrevida mostraram que o melhor prognóstico foi relacionado a idade abaixo de 60 x anos(mediana 496dias; p-valor<0,0001 ; OR 0,2774),paciente com LMA de novo(mediana 264dias; p-valor 0,0103; OR 0,3858).Não houve diferença estatisticamente significante quando comparadas as curvas de sobrevida global com a citogenética, a leucometria maior ou menor de100x10e3/μl, e pacientes que fez ou não TMO. Em conclusão, os resultados obtidos permitiram descrever um perfil das características demográficas e clínicas da LMA no Amazonas, fornecendo ferramentas importantes para um eventual desenvolvimento de sistemas de vigilância. Palavras Chaves. Leucemia mieloide aguda, neoplasias hematológicas.
description ABSTRACT Acute myeloid leukemia (AML) is a neoplasm of progenitor hematopoietic cells that results in loss of the ability to differentiate from the myeloid lineage, leading to insufficient generation of mature blood cells. LMA is a genetically heterogeneous disease, which occurs most commonly in adults, with increasing incidence with increasing age. The diagnosis, according to the World Health Organization - WHO, requires the presence of 20% of blasts in the bone marrow. It is a highly aggressive disease with a still very low survival rate despite chemotherapeutic treatment, with the exception of acute promyelocytic leukemia. The objective of this study is to characterize the clinical and epidemiological profile of AML patients in the State of Amazonas treated in HEMOAM. The study was retrospective, cross- sectional, including all patients diagnosed with AML between January 2013 and December 2017.were 194 patients diagnosed in this period, patients who were not treated exclusively in HEMOAM, and the medical records were not found were excluded. The median age of the population was 46 years, 70,94% were of the non-white race, the distribution between men and women 96:83.The most frequent clinical manifestations were asthenia (65.89%), other associated symptoms (50,28%), fever (48,55%), and bleeding (31,21%). The majority of the population (45.97%) had a performance status according to the ECOG score of (2). The most frequent FAB subtypes found in this work was M5 followed by M3.The median of hemoglobin was 8.4 (4.1 -14) g\/dl, with median VCM 89 (69.10 interval -115.8) fl. The median of the Leukocyte count was 11660\/mm3 and platelet median was 43000\/mm3. Median (%) peripheral blood blasts was 26% and the bone marrow 71%. With respect to the risk cytogenetic (68%) karyotypes are intermediate risk. In immunophenotyping was the most common marker CD13 being expressed (91,51%) of the cases. Of the findings 53,93% expressed aberrant antigens, with CD7 being most expressed in the T lymphoid line and CD19 in the B lymphoid line. A total of 5 patients were classified as acute bifenotypic leukemia (LAB). The most used treatment lineage in the adult was 3+7, in the children was the BFM 2004. A total of 134 patients were submitted to intensive therapy of which 74 entered in remission. Nine (9) of the patients underwent allogeneic hematopoietic stem cell transplantation. The rate of relapse and refractory were raised. The overall survival at 1 year was 31.28%. The death rate was 67.59%. The survival curves showed that better prognosis was related to the age below of 60 years (median 496dias; p-value < 0.0001; OR 0.2774), patient with AML de novo (median 264dias; p-value 0.0103; OR 0.3858).There was no statistically significant difference when comparing overall survival curves with the xii cytogenetics, the leukocytes more or less de100x10e3\/μ l, and patients who did or didn't BMT. Conclusion, the results obtained made it possible to obtain a profile of demographic and clinical features of AML in the Amazon, provide important tools for a possible development of surveillance systems. Keywords. Acute myeloid leukemia, haematological malignancies
publishDate 2019
dc.date.none.fl_str_mv 2019-04-22
2020-03-09
2020-03-09T15:07:37Z
2020-03-09T15:07:37Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/masterThesis
status_str publishedVersion
format masterThesis
dc.identifier.uri.fl_str_mv http://repositorioinstitucional.uea.edu.br//handle/riuea/2131
url http://repositorioinstitucional.uea.edu.br//handle/riuea/2131
dc.language.iso.fl_str_mv por
language por
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dc.rights.driver.fl_str_mv Atribuição-NãoComercial-SemDerivados 3.0 Brasil
http://creativecommons.org/licenses/by-nc-nd/3.0/br/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Atribuição-NãoComercial-SemDerivados 3.0 Brasil
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dc.publisher.none.fl_str_mv Universidade do Estado do Amazonas
Brasil
Programa de Pós-Graduação em Ciências Aplicadas à Hematologia
UEA
publisher.none.fl_str_mv Universidade do Estado do Amazonas
Brasil
Programa de Pós-Graduação em Ciências Aplicadas à Hematologia
UEA
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reponame_str Repositório Institucional da Universidade do Estado do Amazonas (UEA)
collection Repositório Institucional da Universidade do Estado do Amazonas (UEA)
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