%0 Journal Article
%T Mayor Erythropoietic Response after Deferasirox Treatment in a Transfusion-Dependent Anemic Patient with Primary Myelofibrosis
%A Del Corso Lisette
%A Balleari Enrico
%A Arboscello Eleonora
%A Ghio Riccardo
%A Mencoboni Manlio
%A Racchi Omar
%J Case Reports in Hematology
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/520712
%X Primary myelofibrosis (PMF) is a myeloproliferative neoplasm frequently complicated by transfusion dependent anemia. Both anemia and transfusion-dependence are associated with a poor outcome, at least in part because of toxic effects of iron overload (IOL). Iron-chelating therapy (ICT) is increasingly used in order to prevent IOL in this setting. Here, we describe the case of a 73-year-old man affected by PMF and severe transfusion-dependent anemia who experienced a dramatic erythroid response after being treated with deferasirox to prevent IOL. 1. Introduction Anemia is a frequent complication of primary myelofibrosis (PMF), either at presentation or during the course of the disease, with an incidence and diagnosis ranging from 50 to 70% [1]. The presence of anemia in PMF is well known to negatively impact survival, and transfusion dependence has been recently reported as a further negative prognostic factor; both of these variables are included in the more used current PMF prognostic scores [2, 3]. Although severe anemia could per se indicate a more aggressive disease with higher risk of leukemic transformation, the increased mortality in severely anemic PMF patients does not seem to be related entirely to leukemia but also to the negative effects of chronic low hemoglobin levels on cardiovascular system, and, in the heavily transfused patients, it might also be dependent on the systemic damage of the heart and other organs due to iron overload (IOL). IOL is also believed to increase the infective risk of these already frail patients. To limit the toxicity of iron excess, iron-chelating therapy (ICT), although not routinely recommended by current guidelines of PMF management, has been recently increasingly proposed in the management of these patients, when transfusion-dependent anemia occurs. A positive effect from ICT on survival in patients with PMF has been already demonstrated by Leitch et al. [4], and it was mainly attributed to a reduction of toxic effects of IOL. A possible direct effect of ICT in improving erythropoiesis of patients with PMF has also been described, even if in a few cases [5¨C8]. Here, we describe a PMF patient with severe transfusion-dependent anemia in which ICT with deferasirox stunningly restored normal hemoglobin levels. 2. Case Presentation A 73-year-old Caucasian otherwise healthy man came to our outpatient¡¯s clinic in August 2011 because of neutrophil leukocytosis and splenomegaly. Blood counts were as follows: white blood cells (WBC) 28.2 ¡Á 109/L, hemoglobin (Hb) 11.5£¿g/dL, and platelets (Plt) 350 ¡Á 109/L. Physical
%U http://www.hindawi.com/journals/crihem/2013/520712/