Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions.
BACKGROUND
Transfusion therapy is frequently used to prevent morbidity in sickle cell disease (SCD), and subsequent iron overload is common. The objective of this study was to evaluate the current standard of care in monitoring iron overload and related complications in patients with SCD compared to thalassemia (Thal).


METHODS
A cross-sectional study was conducted at 31 hematology clinics in the United States, Canada, or the United Kingdom. Patients who received transfusions with a mean serum ferritin level of least 2000 ng per mL were eligible. A total of 199 patients with SCD (113 female; 24.9 +/- 13.2 years) and 142 with Thal (66 female; 25.8 +/- 8.1 years) were recruited, and data were collected between 2001 and 2003 by interview and medical record review.


RESULTS
Although both groups were recruited on the basis of significant iron overload, the likelihood of performing a liver biopsy for routine iron monitoring was significantly higher (odds ratio [OR], 3.4; 95% confidence interval [CI], 2.2-5.3) in Thal than SCD. Thal patients were also more likely to be screened for iron-related organ injury including an echocardiograph for cardiomyopathy (OR, 2.6; p < 0.001; 95% CI, 1.6-4.2), alanine aminotransferase for liver function (OR, 8.3; CI, 1.05-64.4), and thyroid-stimulating hormone for hypothyroidism (OR, 12.3; CI, 7.0-21.5). For adult SCD patients, those maintained on simple transfusion with a serum ferritin level of greater than 2500 ng per mL were the least likely to have a liver biopsy (p < 0.03).


CONCLUSIONS
These data highlight the unsystematic monitoring of iron and related organ injury in SCD. Until the relationship between iron and related comorbidities is better understood, routine monitoring of iron overload in SCD patients who receive transfusions should be considered a standard part of clinical care.
Department of Hematology, The Children's Hospital & Research Center, Oakland, California, USA. efung@mail.cho.org
Ellen B
EB
Fung
Paul R
PR
Harmatz
Meredith
M
Milet
Vinod
V
Balasa
Samir K
SK
Ballas
James F
JF
Casella
Lee
L
Hilliard
Abdullah
A
Kutlar
Kenneth L
KL
McClain
Nancy F
NF
Olivieri
John B
JB
Porter
Elliott P
EP
Vichinsky
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000628:therapy*; D002170:Canada; D002648:Child; D003430:Cross-Sectional Studies; D017707:Erythrocyte Transfusion / Q000009:adverse effects*; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D019190:Iron Overload / Q000209:etiology / Q000628:therapy*; D008297:Male; D013789:Thalassemia / Q000628:therapy*; D006113:United Kingdom; D014481:United States; D055815:Young Adult
D005293:Ferritins