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Administration of recombinant erythropoietin alone does not improve the phenotype in iron refractory iron deficiency anemia patients.
MedLine Citation:
PMID:  23180434     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Mutations in transmembrane protease, serine 6 (TMPRSS6) cause iron refractory iron deficiency anemia (IRIDA). Parenteral iron administration may slightly improve hemoglobin level but is troublesome for patients. Optimal treatment has yet to be determined. We identified five patients from four independent families displaying the IRIDA picture with truncating biallelic mutations in TMPRSS6, one of which is novel. Liver iron determined by superconducting quantum interference device biosusceptometry ranged from 390 to 720 µg Fe/g wet weight (normal range 100-500; n = 3). Intestinal iron absorption (12 and 32 %, normal range 10-50; n = 2) and 59Fe erythrocyte incorporation after ingestion of 59Fe (57 and 38 %, normal range 70-90; n = 2) were inadequately low for iron-deficient anemic individuals. Baseline serum erythropoietin was elevated or borderline high in four patients. Administration of recombinant human erythropoietin (rhEPO) at up to 273 and 188 U/kg body weight/week alone did not improve anemia or result in a decrease of urinary hepcidin in two individuals. In conclusion, the ability of exogenous rhEPO to increase hemoglobin level appears to be impaired in IRIDA.
Authors:
Kai Lehmberg; Regine Grosse; Martina U Muckenthaler; Sandro Altamura; Peter Nielsen; Hansjörg Schmid; Ulrike Graubner; Florian Oyen; Wolfgang Zeller; Reinhard Schneppenheim; Gritta E Janka
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-11-20
Journal Detail:
Title:  Annals of hematology     Volume:  -     ISSN:  1432-0584     ISO Abbreviation:  Ann. Hematol.     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-11-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9107334     Medline TA:  Ann Hematol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Pediatric Hematology and Oncology, University Medical Center Eppendorf, Martinistrasse 52, 20246, Hamburg, Germany, k.lehmberg@uke.de.
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