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      Tratamento de suporte e quelação de ferro em pacientes com síndromes mielodisplásicas Translated title: Supportive care, tranfusion and chelation therapy for patients with myelodysplastic syndromes

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          Abstract

          As síndromes mielodisplásicas (SMD) são um grupo heterogêneo de distúrbios hematológicos que ocorrem mais freqüentemente em pacientes idosos e que cursa, na maioria dos casos, com anemia crônica dependente de transfusão de hemoderivados. Conseqüentemente, muitos destes pacientes passam a apresentar sobrecarga de ferro, que pode levar a danos teciduais graves. Ambas as terapias, transfusional e de quelação de ferro, quando indicadas, são importantes para manter a sobrevida e a qualidade de vida destes pacientes. A terapia de quelação de ferro está indicada especialmente nos subtipos de SMD com melhor prognóstico e sobrevida longa o suficiente para o desenvolvimento de sobrecarga de ferro com relevância clínica. A terapia de quelação de ferro apresenta algumas limitações relacionadas à necessidade de longo tempo de infusão da deferoxamina, da dificuldade de adesão pelo paciente, bem como da aquisição da bomba de infusão. O uso da deferiprona, que é um quelante oral de ferro, está contra-indicado neste grupo de pacientes, pelo risco de neutropenia e agranulocitose. O deferasirox é um novo quelante oral de ferro em estudo e que poderá, no futuro, ser uma opção adequada para os pacientes com SMD e sobrecarga de ferro. Novos estudos em pacientes com síndromes mielodisplásicas são necessários para melhor estabelecer critérios de diagnóstico da sobrecarga de ferro, bem com da terapia de quelação neste grupo.

          Translated abstract

          Myelodysplastic syndromes (MDSs) are a heterogeneous group of hematological disorders which are more common in the elderly and related to chronic anemia dependent on blood transfusions. Consequently, many of these patients develop iron overload which may lead to severe injury to tissues. Transfusions and chelation therapy, when indicated, are important for survival and to maintain the quality of life. Chelation therapy is indicated especially for MDS subtypes with a better prognosis and a sufficiently long survival to develop clinically relevant iron overload. Chelation therapy presents with some limitations in particular the long time required for deferoxamine infusion and the difficulties of patients to comply with treatment and to acquire an infusion pump. The clinical use of deferiprone, an oral chelator, is not indicated for MDS patients because of the risk of neutropenia and agranulocytosis. Deferasirox is a new oral chelator currently under clinical development that will probably be, in the future, an adequate option for MDS patients with iron overload. Additional studies in MDS patients are necessary to establish better diagnostic and chelation therapy criteria.

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          Most cited references12

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          Monitoring and treatment of iron overload: state of the art and new approaches.

          J Porter (2005)
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            • Record: found
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            Evidence - and consensus - based practice guidelines for the therapy of primary mielodysplastic syndromes: A statement from the Italian Society of Hematology

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              • Record: found
              • Abstract: not found
              • Article: not found

              Secondary hemochromatosis in polytransfused patients with myelodysplastic syndromes

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                Author and article information

                Contributors
                Role: ND
                Journal
                rbhh
                Revista Brasileira de Hematologia e Hemoterapia
                Rev. Bras. Hematol. Hemoter.
                Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (São Paulo )
                1806-0870
                September 2006
                : 28
                : 3
                : 204-209
                Affiliations
                [1 ] Hospital Brigadeiro Brazil
                [2 ] Laboratório Diagnósticos da América
                Article
                S1516-84842006000300011
                10.1590/S1516-84842006000300011
                7d78e8ce-fc16-4575-8929-cd4bc2a0d283

                http://creativecommons.org/licenses/by/4.0/

                History
                Product

                SciELO Brazil

                Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=1516-8484&lng=en
                Categories
                HEMATOLOGY
                MEDICINE, RESEARCH & EXPERIMENTAL

                Medicine,Hematology
                Myelodysplastic syndromes,supportive care,transfusion therapy,iron overload,chelation therapy,Síndromes mielodisplásicas,tratamento de suporte,terapia transfusional,sobrecarga de ferro,quelação de ferro

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