Transplante de medula óssea em pacientes com anemia falciforme no Brasil: uma revisão de literatura
Sickle-cell disease are the most common monogenic disorders in the world, being the sickle cell anemia, the most prevalent. People with sickle cell anemia are homozygous for hemoglobin S (Hb SS), which causes erythrocytes falcization when in deoxygenated state. This characteristic can lead to vessel...
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Anemia Falciforme Tratamento Transplante de Células Tronco Hematopoiéticas |
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Anemia Falciforme Tratamento Transplante de Células Tronco Hematopoiéticas Castro, Fernanda Dantas de Transplante de medula óssea em pacientes com anemia falciforme no Brasil: uma revisão de literatura |
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Sickle-cell disease are the most common monogenic disorders in the world, being the sickle cell anemia, the most prevalent. People with sickle cell anemia are homozygous for hemoglobin S (Hb SS), which causes erythrocytes falcization when in deoxygenated state. This characteristic can lead to vessels obstruction, causing vaso occlusive crises, very common in this disease, and episodes of intense pain in the patients. Estimates suggest that every year approximately 250,000 infants are born worldwide with sickle cell anemia. Therefore, due to the high prevalence and major impact on the morbidity and mortality of affected population, concentrating higher mortality rates in the first two years of age, it was recognized by the World Health Organization as a serious global health problem, and, in Brazil, in 2001, it was included as a part of neonatal screening program, allowing an early diagnosis of the disease and the adoption of adequate prophylactic measures and patient follow-up. Conventional treatment for sickle cell anemia includes blood transfusions, analgesics and hydroxycarbamide, besides the prophylactic measures such as vaccination. Even though they have improved the survival rates of these patients, they have their adverse effects and offer no possibility of cure. Thus, allogeneic hematopoietic stem cell transplantation is the only curative treatment for sickle cell anemia. Although there are indications that restrict transplantation to certain patients and complications related to this procedure, in addition to the difficulty of finding a compatible donor, studies show that transplantation can increase patients' disease-free survival rate by up to 95%. Despite the few existing studies related to bone marrow transplantation in patients with sickle cell anemia in Brazil, this therapy has been shown to be promising and it is estimated that further studies will be done to make it even more feasible |
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Medeiros, Karina Carla de Paula |
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Medeiros, Karina Carla de Paula Castro, Fernanda Dantas de |
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bachelorThesis |
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Castro, Fernanda Dantas de |
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Castro, Fernanda Dantas de |
| title |
Transplante de medula óssea em pacientes com anemia falciforme no Brasil: uma revisão de literatura |
| title_short |
Transplante de medula óssea em pacientes com anemia falciforme no Brasil: uma revisão de literatura |
| title_full |
Transplante de medula óssea em pacientes com anemia falciforme no Brasil: uma revisão de literatura |
| title_fullStr |
Transplante de medula óssea em pacientes com anemia falciforme no Brasil: uma revisão de literatura |
| title_full_unstemmed |
Transplante de medula óssea em pacientes com anemia falciforme no Brasil: uma revisão de literatura |
| title_sort |
transplante de medula óssea em pacientes com anemia falciforme no brasil: uma revisão de literatura |
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Universidade Federal do Rio Grande do Norte |
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2018 |
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https://repositorio.ufrn.br/handle/123456789/43143 |
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AT castrofernandadantasde transplantedemedulaosseaempacientescomanemiafalciformenobrasilumarevisaodeliteratura |
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1773959414500294656 |
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ri-123456789-431432021-10-06T11:13:51Z Transplante de medula óssea em pacientes com anemia falciforme no Brasil: uma revisão de literatura Castro, Fernanda Dantas de Medeiros, Karina Carla de Paula Fernandes, Thales Allyrio Araújo de Medeiros Anemia Falciforme Tratamento Transplante de Células Tronco Hematopoiéticas Sickle-cell disease are the most common monogenic disorders in the world, being the sickle cell anemia, the most prevalent. People with sickle cell anemia are homozygous for hemoglobin S (Hb SS), which causes erythrocytes falcization when in deoxygenated state. This characteristic can lead to vessels obstruction, causing vaso occlusive crises, very common in this disease, and episodes of intense pain in the patients. Estimates suggest that every year approximately 250,000 infants are born worldwide with sickle cell anemia. Therefore, due to the high prevalence and major impact on the morbidity and mortality of affected population, concentrating higher mortality rates in the first two years of age, it was recognized by the World Health Organization as a serious global health problem, and, in Brazil, in 2001, it was included as a part of neonatal screening program, allowing an early diagnosis of the disease and the adoption of adequate prophylactic measures and patient follow-up. Conventional treatment for sickle cell anemia includes blood transfusions, analgesics and hydroxycarbamide, besides the prophylactic measures such as vaccination. Even though they have improved the survival rates of these patients, they have their adverse effects and offer no possibility of cure. Thus, allogeneic hematopoietic stem cell transplantation is the only curative treatment for sickle cell anemia. Although there are indications that restrict transplantation to certain patients and complications related to this procedure, in addition to the difficulty of finding a compatible donor, studies show that transplantation can increase patients' disease-free survival rate by up to 95%. Despite the few existing studies related to bone marrow transplantation in patients with sickle cell anemia in Brazil, this therapy has been shown to be promising and it is estimated that further studies will be done to make it even more feasible As doenças falciformes são as desordens monogênicas mais comuns em todo o mundo, sendo a mais prevalente destas, a anemia falciforme, que resulta da homozigose da hemoglobina S (Hb SS), a qual provoca deformação nos eritrócitos que passam a adquirir um formato de foice, quando desoxigenados. Tal característica favorece a obstrução de vasos, gerando as crises vaso oclusivas, muito frequentes nessa doença, e que provocam crises de dor intensa no paciente. Estima-se que, a cada ano, nasçam aproximadamente 250 mil crianças com anemia falciforme em todo o mundo. Desse modo, devido à alta prevalência e por ter grande impacto na morbimortalidade da população afetada, concentrando maiores taxas de mortalidade nos dois primeiros anos de idade, passou a ser reconhecida pela Organização Mundial da Saúde como um grave problema de saúde pública mundial. No Brasil, em 2001, passou a ser inclusa no Programa de Triagem Neonatal, possibilitando o diagnóstico precoce da doença. Os principais tratamentos convencionais para a anemia falciforme incluem transfusões sanguíneas, analgésicos e hidroxiuréia, além de medidas profiláticas, como a vacinação. Ainda que estas terapias proporcionem melhoras nas taxas de sobrevida desses pacientes, possuem seus efeitos adversos e não oferecem possibilidade de cura aos mesmos. Dessa forma, o transplante alogênico de células-tronco hematopoiéticas constitui-se como o único tratamento curativo para a anemia falciforme. Embora haja indicações que restrinjam o transplante a determinados pacientes e complicações relacionadas a esse procedimento, além da dificuldade de se encontrar doador compatível, estudos mostram que o transplante pode aumentar em até 95% a taxa de sobrevida livre de doença dos pacientes. Apesar de ainda haver poucos estudos existentes sobre o transplante de medula óssea em pacientes com anemia falciforme no Brasil, essa terapêutica tem se mostrado promissora e estima-se que novos estudos sejam feitos para viabilizar ainda mais sua realização 2018-01-11T12:13:42Z 2021-10-06T11:13:51Z 2018-01-11T12:13:42Z 2021-10-06T11:13:51Z 2017-12-05 bachelorThesis 2013084010 CASTRO, Fernanda Dantas de. Transplante de medula óssea em pacientes com anemia falciforme no Brasil: uma revisão de literatura. 2017. 47 f. Trabalho de Conclusão de Curso (Graduação em Biomedicina) - Departamento de Biomedicina, Centro de Biociências, Universidade Federal do Rio Grande do Norte, Natal, 2017. https://repositorio.ufrn.br/handle/123456789/43143 pt_BR openAccess application/pdf Universidade Federal do Rio Grande do Norte Brasil UFRN Biomedicina |