Abstract
We report three cases of myelodysplastic syndrome (MDS), refractory anemia with ringed sideroblasts (RARS), who responded to the anti-malarial chloroquine in combination with recombinant erythropoietin (darbepoetin alfa). All three patients either remained or achieved transfusion independence and increased their hemoglobin level by at least 1 gm/dl. Responses occurred rapidly, becoming clinically noticeable within the first 4-6 weeks from start of therapy. All patients were followed for at least a 6 months period during which they have remained transfusion independent. Chloroquine affects multiple cellular pathways, but the precise mechanism of its action in RARS patients is unclear.
Highlights
Refractory anemia with ringed sideroblasts (RARS) is a common, low grade subtype of myelodysplastic syndromes (MDS) characterized by the presence of erythroblasts showing iron filled mitochondria around the nucleus [1]
Two distinct types of RARS are recognized on the basis of finding refractory anemia with uni-lineage dysplasia (RARS) or refractory cytopenias with multi-lineage dysplasia and ringed sideroblasts (RCMD-RS)
Erythropoiesis stimulating agents (ESA) are less effective for the anemia of RARS [3] but responses improve when combined with granulocyte colony stimulating factor (G-CSF)
Summary
Three Cases of Refractory Anemia with Ringed Sideroblasts Who Responded to Chloroquine.
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