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Absence of cardiac siderosis by MRI T2* despite transfusion burden, hepatic and serum iron overload in Lebanese patients with sickle cell disease

Show simple item record Inati A. Musallam K.M. Wood J.C. Sheikh-Taha M. Daou L. Taher A.T.
dc.contributor.editor Dec-2009 2017-10-05T15:41:04Z 2017-10-05T15:41:04Z 2009
dc.identifier 10.1111/j.1600-0609.2009.01345.x
dc.identifier.issn 09024441
dc.description.abstract Background: The use of magnetic resonance imaging (MRI) to detect organ-specific iron overload is becoming increasingly common. Although hepatic iron overload has been recognized in patients with sickle cell disease (SCD), cardiac iron deposition has only been examined in a few reports. Methods: This was a cross-sectional study of 23 patients with SCD. Patient charts were reviewed and data collected for drug use, total lifetime transfusions (TLT), transfusion rate (TR), status of the spleen, and comorbid illnesses or infections. Blood samples were obtained for assessment of hemoglobin, serum ferritin, non-transferrin-bound iron (NTBI), and liver enzyme levels. Doppler echocardiography was performed to detect pulmonary hypertension (PHT) and assess left ventricular ejection fraction. Cardiac iron levels were measured by MRI T2*. Direct determination of liver iron concentration (LIC) was performed using R2 MRI. In this study, cardiac T2* 20 ms was considered normal. Results: The mean age was 24.4 ± 9.7 yr, with a male to female ratio of 15:8. A total of 9 (49.9percent) patients were splenectomized. The mean TR was 14.1 ± 13.2 Units-yr, and the mean hemoglobin level was 9.0 g-dL. PHT was detected in 6 (27.3percent) patients, but none had evidence of heart failure. The mean serum ferritin, LIC, and NTBI levels were 997.7 ng-mL, 4.6 mg Fe-g dw, and 1.1 ± 2.2, respectively. TR was a much better predictor of iron burden (LIC, ferritin, NTBI) than TLT. In fact, TR less than 10 Units-yr did not produce significant iron overload reflecting spontaneous losses as high as 0.11 mg-kg-d. None of the patients had evidence of cardiac iron overload (mean cardiac T2* = 37.3 ± 6.2 ms; range: 21.9-46.8 ms). There was also no statistically significant correlation between cardiac T2* values and any of the study variables. Conclusion: Our study demonstrates that TR is a stronger predictor of iron overload than TLT. It also confirms cardiac sparing in patients with SCD, even in subjects with significant transfusion burden, systemic and hepatic iron overload. © 2009 John Wiley and Sons A-S.
dc.format.extent Pages: (565-571)
dc.language English
dc.publisher MALDEN
dc.relation.ispartof Publication Name: European Journal of Haematology; Publication Year: 2009; Volume: 83; no. 6; Pages: (565-571);
dc.source Scopus
dc.title Absence of cardiac siderosis by MRI T2* despite transfusion burden, hepatic and serum iron overload in Lebanese patients with sickle cell disease
dc.type Article
dc.contributor.affiliation Inati, A., Division of Pediatric Hematology and Oncology, Children's Center for Cancer and Blood Diseases, Rafik Hariri University Hospital, Bir Hasan, Beirut, Lebanon, Nini Hospital, Tripoli, Lebanon, Balamand University, Balamand, Lebanon
dc.contributor.affiliation Musallam, K.M., Department of Internal Medicine, Beirut Medical Center, American University, Beirut, Lebanon
dc.contributor.affiliation Wood, J.C., Divisions of Pediatric Cardiology and Radiology, University of Southern California, Children's Hospital Los Angeles, Los Angeles, CA, United States
dc.contributor.affiliation Sheikh-Taha, M., School of Pharmacy, Lebanese American University, Byblos, Lebanon
dc.contributor.affiliation Daou, L., Divisions of Pediatric Cardiology, Hotel Dieu de France University Hospital, Beirut, Lebanon
dc.contributor.affiliation Taher, A.T., Department of Internal Medicine, Beirut Medical Center, American University, Beirut, Lebanon
dc.contributor.authorAddress Inati, A.; Division of Pediatric Hematology and Oncology, Children's Center for Cancer and Blood Diseases, Rafik Hariri University Hospital, Bir Hasan, Beirut, Lebanon; email:
dc.contributor.authorCorporate University: American University of Beirut Medical Center; Faculty: Faculty of Medicine; Department: Internal Medicine;
dc.contributor.authorDepartment Internal Medicine
dc.contributor.faculty Faculty of Medicine
dc.contributor.authorInitials Inati, A
dc.contributor.authorInitials Musallam, KM
dc.contributor.authorInitials Wood, JC
dc.contributor.authorInitials Sheikh-Taha, M
dc.contributor.authorInitials Daou, L
dc.contributor.authorInitials Taher, AT
dc.contributor.authorReprintAddress Inati, A (reprint author), Rafik Hariri Univ Hosp, Div Pediat Hematol and Oncol, Childrens Ctr Canc and Blood Dis, Beirut, Lebanon.
dc.contributor.authorUniversity American University of Beirut Medical Center
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dc.description.citedCount 19
dc.description.citedTotWOSCount 18
dc.description.citedWOSCount 18
dc.format.extentCount 7
dc.identifier.coden EJHAE
dc.identifier.pubmedID 19737308
dc.identifier.scopusID 70449499193
dc.publisher.address COMMERCE PLACE, 350 MAIN ST, MALDEN 02148, MA USA
dc.relation.ispartOfISOAbbr Eur. J. Haematol.
dc.relation.ispartOfIssue 6
dc.relation.ispartofPubTitle European Journal of Haematology
dc.relation.ispartofPubTitleAbbr Eur. J. Haematol.
dc.relation.ispartOfVolume 83
dc.source.ID WOS:000271631300009
dc.type.publication Journal
dc.subject.otherAuthKeyword Heart
dc.subject.otherAuthKeyword Iron overload
dc.subject.otherAuthKeyword Magnetic resonance imaging
dc.subject.otherAuthKeyword Sickle cell disease
dc.subject.otherAuthKeyword Transfusion
dc.subject.otherChemCAS ferritin, 9007-73-2
dc.subject.otherChemCAS hemoglobin, 9008-02-0
dc.subject.otherChemCAS iron, 14093-02-8, 53858-86-9, 7439-89-6
dc.subject.otherChemCAS Ferritins, 9007-73-2
dc.subject.otherChemCAS Hemoglobins
dc.subject.otherChemCAS Iron, 7439-89-6
dc.subject.otherIndex ferritin
dc.subject.otherIndex hemoglobin
dc.subject.otherIndex iron
dc.subject.otherIndex liver enzyme
dc.subject.otherIndex adult
dc.subject.otherIndex article
dc.subject.otherIndex blood sampling
dc.subject.otherIndex cardiac siderosis
dc.subject.otherIndex clinical article
dc.subject.otherIndex clinical assessment
dc.subject.otherIndex clinical evaluation
dc.subject.otherIndex comorbidity
dc.subject.otherIndex correlation coefficient
dc.subject.otherIndex Doppler echocardiography
dc.subject.otherIndex female
dc.subject.otherIndex ferritin blood level
dc.subject.otherIndex heart left ventricle ejection fraction
dc.subject.otherIndex high performance liquid chromatography
dc.subject.otherIndex human
dc.subject.otherIndex infection
dc.subject.otherIndex iron blood level
dc.subject.otherIndex iron overload
dc.subject.otherIndex Lebanon
dc.subject.otherIndex liver
dc.subject.otherIndex male
dc.subject.otherIndex medical record review
dc.subject.otherIndex nuclear magnetic resonance imaging
dc.subject.otherIndex priority journal
dc.subject.otherIndex pulmonary hypertension
dc.subject.otherIndex sickle cell anemia
dc.subject.otherIndex siderosis
dc.subject.otherIndex spleen
dc.subject.otherIndex splenectomy
dc.subject.otherIndex transfusion
dc.subject.otherIndex Adolescent
dc.subject.otherIndex Adult
dc.subject.otherIndex Anemia, Sickle Cell
dc.subject.otherIndex Blood Transfusion
dc.subject.otherIndex Child
dc.subject.otherIndex Child, Preschool
dc.subject.otherIndex Cross-Sectional Studies
dc.subject.otherIndex Female
dc.subject.otherIndex Ferritins
dc.subject.otherIndex Hemoglobins
dc.subject.otherIndex Humans
dc.subject.otherIndex Iron
dc.subject.otherIndex Iron Overload
dc.subject.otherIndex Lebanon
dc.subject.otherIndex Liver
dc.subject.otherIndex Male
dc.subject.otherIndex Middle Aged
dc.subject.otherIndex Myocardium
dc.subject.otherIndex Sampling Studies
dc.subject.otherIndex Splenectomy
dc.subject.otherIndex Young Adult
dc.subject.otherKeywordPlus BETA-THALASSEMIA
dc.subject.otherKeywordPlus MYOCARDIAL IRON
dc.subject.otherKeywordPlus CHELATION-THERAPY
dc.subject.otherKeywordPlus LIVER IRON
dc.subject.otherKeywordPlus DEFEROXAMINE
dc.subject.otherKeywordPlus HEART
dc.subject.otherKeywordPlus CARDIOMYOPATHY
dc.subject.otherKeywordPlus DEFERASIROX
dc.subject.otherKeywordPlus DYSFUNCTION
dc.subject.otherWOS Hematology

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