Iron Metabolism, Biomarkers, Iron Overload and Chelation: Consensus in Myelodysplastic Syndromes

Abstract

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 recurrent 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, hematopoietic growth factors, antifibrinolytic agents and chelators.
Chelation therapy should be considered in patients transfused 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.