Hans Tesch1, Christian Ihling. 1. Onkologie Bethanien, Frankfurt/M., Germany. hans.tesch@chop-studien.de
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. CASE REPORT: 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. CONCLUSION: Iron chelation treatment with deferasirox resulted in an improvement of hematologic parameters and loss of transfusion dependency.
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. CASE REPORT: 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. CONCLUSION:Iron chelation treatment with deferasirox resulted in an improvement of hematologic parameters and loss of transfusion dependency.