Metabolismo do ferro, biomarcadores, sobrecarga de ferro e quelação: recomendações em síndromes mielodisplásicas

Myelodysplastic syndromes (MDS) are a diverse group of myeloid neoplasias, characterized by cytopenias stemming from ineffective hematopoiesis and by an increased risk of progression to acute myeloid leukemia. They affect 5/100 000 people per year in Portugal, among which more than 60% will become transfusion-dependent during the course of the disease. Cytopenias may lead to significant morbidity and their correct management is the key to maintain these patients’ quality of life. This document gathers the result of the debate promoted by the Anemia Working Group Portugal with experts on the management of patients with lower risk MDS, addressing the medium- and long-term effects of recur-rent transfusion and iron overload. For the diagnosis of MDS, the group recommends the use of World Health Organization classification published in 2016. The International Prognosis Scoring System score is still the reference score to define both the prognosis and the therapeutic strategy. Support therapy is a fundamental cornerstone in the management of MDS patients at any stage of the disease, and includes antibiotics, transfusion of erythrocytes and platelets, hemato-poietic growth factors, antifibrinolytic agents and chelators. Chelation therapy should be considered in patients transused with ≥ 20 units of erythrocytes or with serum ferritin ≥1000 ng/mL. Patients who are candidates to chelation should be submitted to periodic magnetic resonance imaging to the liver and the pancreas. Therapeutic options should take into account the individual characteristics of the disease for every patient and preserve the quality of live, in accordance with international guidelines.