Iron burden and liver fibrosis decrease during a long-term phlebotomy program and iron chelating treatment after bone marrow transplantation.
In this retrospective study, we report the results of the association of a combined phlebotomy program and chelation in hereditary sideroblastic anemia (HSA) to reduce iron overload after bone marrow transplantation (BMT). A male HSA patient, not responding to pyridoxine treatment, was submitted to successful allogeneic BMT. As there was a persistence of a tissue iron overload, a regular phlebotomy program was started followed by chelation. A significant decrease of iron burden was obtained using a combined treatment with deferoxamine (DFO) and deferiprone (L1) in addition to the phlebotomy program. A 10-year follow-up shows a marked decrease in the concentration of serum ferritin, non-transferrin-bound iron (NTBI), liver iron and normal hemoglobin (Hb), which allows the patient to reach and maintain a good quality of life.
Meo
Anna
A
Paediatric Department, "G. Martino" Policlinico University of Messina, Messina, Italy. ameo@unime.it
Ruggeri
Annalisa
A
La Rosa
Maria A
MA
Zanghì
Laura
L
Morabito
Nancy
N
Duca
Lorena
L
D000293:Adolescent; D000756:Anemia, Sideroblastic / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D015415:Biomarkers / Q000097:blood; D016026:Bone Marrow Transplantation; D015913:Chelation Therapy; D003131:Combined Modality Therapy; D000077543:Deferiprone; D003676:Deferoxamine / Q000627:therapeutic use; D005500:Follow-Up Studies; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D019190:Iron Overload / Q000150:complications / Q000628:therapy*; D008103:Liver Cirrhosis / Q000150:complications / Q000628:therapy*; D008297:Male; D018962:Phlebotomy; D011728:Pyridones / Q000627:therapeutic use; D012189:Retrospective Studies
D015415:Biomarkers; D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D003676:Deferoxamine