Long-term hematological response in a patient with 5q- syndrome after suspension of lenalidomide therapy and further improvement with deferasirox therapy

which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Long-term hematological response in a patient with 5q-syndrome after suspension of lenalidomide therapy and further improvement with deferasirox therapy TO THE EDITOR: Anemia and its related clinical effects, including dependence on red blood cells (RBC) transfusions, are common findings in patients affected by myelodysplastic syndromes (MDS) with isolated chromosome 5q deletion (5q-). However, in recent years, the use of lenalidomide in the management of anemia related to 5q-syndrome has significantly changed the clinical scenario in this specific setting [1]. Several reports have described durable erythroid responses and the achievement of transfusion independence in patients with 5q-syndrome who were treated with lenali-domide despite the discontinuation of this agent and the persistence of the underlying malignant clone [1-4]. In addition , both anemia and transfusion are paradoxically associated with organ injury [5], for which iron chelation therapy (ICT) plays an expanding role in the global management of transfusion-dependent patients with MDS. ICT with de-ferasirox has been associated with hematological improvement and achievement of transfusion independence in some patients with MDS [6, 7], and a potential synergistic effect between deferasirox and lenalidomide has been suggested [4]. Here we describe the unusual outcome of a patient with 5q-syndrome who experienced an early and long-lasting response to lenalidomide despite discontinuation of therapy ; interestingly, the hematological and cytogenetic responses improved during treatment with deferasirox, which was given as a single agent on a long-term basis after the suspension of lenalidomide therapy. Using the International Prognostic Scoring System [8], low-risk MDS typical of 5q-syndrome was diagnosed in a 69-year-old woman in July 2007. Apart from well-controlled arterial hypertension and mild kidney failure, the patient's previous pathological history was unremarkable. Complete blood cell count revealed severe macrocytic anemia (hemoglobin [Hb] level, 66 g/L; mean corpuscular volume , 121 fL; reticulocyte count, 0.2%) and thrombocytosis (platelet count, 681×10 9 /L). Total and differential white blood cells (WBC) counts were within normal limits. All possible pathologies potentially mimicking this framework were ruled out. In particular, JAK2 V617F mutation analysis was negative, and hepatic diseases, hypovitaminosis, and hemolytic disorders were excluded. The common parameters regarding iron metabolism were within normal limits; the baseline serum ferritin level was 137 mg/L. Examination of bone marrow (BM) showed prominent erythroid dysplasia and an increased number of hypolobular megakaryocytes without blasts. Cytogenetic analysis revealed isolated del …