title: Serum ferritin underestimates liver iron concentration in transfusion independent thalassemia patients as compared to regularly transfused thalassemia and sickle cell patients.
abstract: Serum ferritin (SF) and liver iron concentration (LIC), as measured by SQUID biosusceptometry, were assessed in a convenience sample of transfusion independent thalassemia patients (nTx-Thal, n=26), regularly transfused thalassemia (Tx-Thal, n=89), or sickle cell patients (SCD, n=45) to investigate the severity of iron overload and the relationship between SF and LIC in nTx-Thal compared to SCD and Tx-Thal. SF correlated with LIC (RS=0.53, P<0.001), but was found to be a poor predictor for LIC. SF was significantly lower (P<0.001) in nTx-Thal patients than in other groups, despite similar LIC values. The SF-to-LIC ratio was significantly lower in nTx-Thal compared to Tx-Thal and SCD patients (median of 0.32, 0.87, and 1.2, respectively: P<0.001). Due to underestimation of LIC by ferritin levels, chelation treatment may be delayed or misdirected in patients with thalassemia intermedia.
authors.affiliation: Children's Hospital & Research Center Oakland, Oakland, California 94609, USA, and University Medical Center Hamburg-Eppendorf, Hamburg, Germany. zpakbaz@mail.cho.org
authors.forename: Zahra
authors.initials: Z
authors.lastname: Pakbaz
authors.forename: Roland
authors.initials: R
authors.lastname: Fischer
authors.forename: Ellen
authors.initials: E
authors.lastname: Fung
authors.forename: Peter
authors.initials: P
authors.lastname: Nielsen
authors.forename: Paul
authors.initials: P
authors.lastname: Harmatz
authors.forename: Elliott
authors.initials: E
authors.lastname: Vichinsky
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000628:therapy; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D005260:Female; D005293:Ferritins / Q000097:blood*; D006801:Humans; D019190:Iron Overload / Q000175:diagnosis*; D008099:Liver / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D013789:Thalassemia / Q000097:blood* / Q000628:therapy
chemical_list: D005293:Ferritins