title: Complete hematopoietic recovery after continuous iron chelation therapy in a patient with severe aplastic anemia with secondary hemochromatosis.
abstract: A 16-yr-old male patient with hemochromatosis due to multiple packed red blood cell transfusions was referred to our emergency center for the treatment of severe aplastic anemia and dyspnea. He was diagnosed with aplastic anemia at 11-yr of age. He had received continuous transfusions because an HLA-matched marrow donor was unavailable. Following a continuous, approximately 5-yr transfusion, he was noted to develop hemochromatosis. He had a dilated cardiomyopathy and required diuretics and digitalis, multiple endocrine and liver dysfunction, generalized bleeding, and skin pigmentation. A total volume of red blood cell transfusion before deferoxamine therapy was about 96,000 mL. He received a regular iron chelation therapy (continuous intravenous infusion of deferoxamine, 50 mg/kg/day for 5 days q 3-4 weeks) for approximately seven years after the onset of multiple organ failures. His cytopenia and organ dysfunctions began to be gradually recovered since about 2002, following a 4-yr deferoxamine treatment. He showed completely normal ranges of peripheral blood cell counts, heart size, and liver function two years ago. He has not received any transfusions for the last four years. This finding suggests that a continuous deferoxamine infusion may play a role in the immune regulation in addition to iron chelation effect.
authors.affiliation: Division of Hematology and Oncology, Our Lady of Mercy Hospital, The Catholic University of Korea, 665 Bupyeng 6-dong, Bupyeng-gu, Incheon, Korea. hellenpark@hanmail.net
authors.forename: Soo-Jeong
authors.initials: SJ
authors.lastname: Park
authors.forename: Chi-Wha
authors.initials: CW
authors.lastname: Han
mesh_terms: D000293:Adolescent; D000741:Anemia, Aplastic / Q000473:pathology / Q000628:therapy*; D015913:Chelation Therapy / Q000379:methods*; D003676:Deferoxamine / Q000627:therapeutic use; D017707:Erythrocyte Transfusion; D006432:Hemochromatosis / Q000150:complications* / Q000628:therapy; D006801:Humans; D007107:Immune System; D007501:Iron / Q000627:therapeutic use*; D007502:Iron Chelating Agents / Q000627:therapeutic use; D008297:Male; D013902:Radiography, Thoracic / Q000379:methods; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007502:Iron Chelating Agents; D007501:Iron; D003676:Deferoxamine