Document Detail


Erythrocytapheresis for chronically transfused children with sickle cell disease: an effective method for maintaining a low hemoglobin S level and reducing iron overload.
MedLine Citation:
PMID:  10540366     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
S T Singer; K Quirolo; K Nishi; E Hackney-Stephens; C Evans; E P Vichinsky
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Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Journal of clinical apheresis     Volume:  14     ISSN:  0733-2459     ISO Abbreviation:  J Clin Apher     Publication Date:  1999  
Date Detail:
Created Date:  1999-12-21     Completed Date:  1999-12-21     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  8216305     Medline TA:  J Clin Apher     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  122-5     Citation Subset:  IM    
Copyright Information:
Copyright 1999 Wiley-Liss, Inc.
Affiliation:
Division of Hematology/Oncology, Children's Hospital Oakland, Oakland, CA 94609-1809, USA.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Anemia, Sickle Cell / therapy*
Blood Transfusion / adverse effects*
Child
Cytapheresis / methods*
Erythroid Precursor Cells*
Female
Hemoglobin, Sickle / metabolism*
Humans
Iron Chelating Agents / therapeutic use
Iron Overload / therapy*
Male
Treatment Outcome
Grant Support
ID/Acronym/Agency:
HL-20985/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/Hemoglobin, Sickle; 0/Iron Chelating Agents

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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