title: Loss of transfusion dependency following deferasirox treatment of iron overload in a woman with myelofibrosis and spherocytosis - a case report.
abstract: BACKGROUND
  Many patients with primary myelofibrosis (PMF), a myeloproliferative neoplasm, become transfusion dependent due to anemia. In these patients, transfusion dependency is associated with shortened survival.
  
  
  METHODS
  We report on a 67-year-old woman who was diagnosed with PMF. Cytogenetic testing revealed a karyotype 46,XX, del(20)(q11.2), consistent with a myeloproliferative disorder. Although offered to her because of severe anemia and leukocytosis, the patient did not consent to allogeneic hematopoietic stem cell transplantation. After having received a cumulative number of more than 70 packed red blood cell transfusions, iron chelation therapy with deferiprone was initiated to treat iron overload and switched to deferasirox after 3 months. Within 2 months of deferasirox treatment, serum ferritin concentrations were significantly reduced, the patient lost transfusion dependency for 17 months, and normal hemoglobin concentrations were recovered.
  
  
  CONCLUSIONS
  Iron chelation treatment with deferasirox resulted in an improvement of hematologic parameters and loss of transfusion dependency.
authors.affiliation: Onkologie Bethanien, Frankfurt/M., Germany. hans.tesch@chop-studien.de
authors.forename: Hans
authors.initials: H
authors.lastname: Tesch
authors.forename: Christian
authors.initials: C
authors.lastname: Ihling
mesh_terms: D000368:Aged; D001565:Benzoates / Q000627:therapeutic use*; D001803:Blood Transfusion / Q000379:methods*; D000077588:Deferasirox; D005260:Female; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000150:complications* / Q000188:drug therapy*; D055728:Primary Myelofibrosis / Q000150:complications* / Q000188:drug therapy; D013103:Spherocytosis, Hereditary / Q000150:complications* / Q000188:drug therapy; D016896:Treatment Outcome; D014230:Triazoles / Q000627:therapeutic use*
chemical_list: D001565:Benzoates; D007502:Iron Chelating Agents; D014230:Triazoles; D000077588:Deferasirox