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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| Formato: | bachelorThesis |
| Idioma: | pt_BR |
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Universidade Federal do Rio Grande do Norte
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| Endereço do item: | https://repositorio.ufrn.br/handle/123456789/43143 |
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| Resumo: | 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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