title: Erythrocytapheresis for chronically transfused children with sickle cell disease: an effective method for maintaining a low hemoglobin S level and reducing iron overload.
abstract: Cerebrovascular accident (CVA) is a major complication of sickle cell disease during childhood. Long-term transfusion reduces the hemoglobin S level and generally prevents recurrent stroke, but it also results in progressive iron overload that requires regular chelation therapy. Erythrocytapheresis offers an alternative approach aimed at reducing the iron accumulation. We reviewed the results of erythrocytapheresis in eight sickle cell patients (mean age of 12.1 years) at high risk for a first or recurrent stroke. They were maintained at the standard pre-transfusion hemoglobin S (Hb S) level of 30%. Over an average of 9 months of erythrocytapheresis, none of the patients developed complications related to the procedure or to the increased blood use. Ferritin levels decreased by a mean of 26.5% in all patients. When evaluating the ferritin level in five patients, who remained on chelation therapy with deferoxamine (DFO), the level dropped by a mean of 32%. The levels remained stable in the three patients who were not on DFO. The procedure is safe and effective in reducing iron overload and can obviate the need for chelation therapy, even when the target Hb S is maintained at the standard 30% range.
authors.affiliation: Division of Hematology/Oncology, Children's Hospital Oakland, Oakland, CA 94609-1809, USA.
authors.forename: S T
authors.initials: ST
authors.lastname: Singer
authors.forename: K
authors.initials: K
authors.lastname: Quirolo
authors.forename: K
authors.initials: K
authors.lastname: Nishi
authors.forename: E
authors.initials: E
authors.lastname: Hackney-Stephens
authors.forename: C
authors.initials: C
authors.lastname: Evans
authors.forename: E P
authors.initials: EP
authors.lastname: Vichinsky
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000628:therapy*; D002648:Child; D016238:Cytapheresis / Q000379:methods*; D015672:Erythroid Precursor Cells; D005260:Female; D006451:Hemoglobin, Sickle / Q000378:metabolism*; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000628:therapy*; D008297:Male; D065227:Transfusion Reaction; D016896:Treatment Outcome
chemical_list: D006451:Hemoglobin, Sickle; D007502:Iron Chelating Agents