Assessment of iron stores in children with transfusion siderosis by biomagnetic liver susceptometry

• Published in: American journal of hematology Vol. 60; no. 4; pp. 289 - 299
• Main Authors: Fischer, Roland, Tiemann, Christian D., Engelhardt, Rainer, Nielsen, Peter, Dürken, Matthias, Gabbe, Erich E., Janka, Gritta E.
• Format: Journal Article
• Language: English
• Published: New York John Wiley & Sons, Inc 01-04-1999; Wiley-Liss
• Subjects: Adolescent; Adult; Animals; beta-Thalassemia - therapy; Biological and medical sciences; biomagnetometry; chelation therapy; Child; Child, Preschool; children; Ferritins - blood; Hematology; Humans; Intestines - chemistry; Investigative techniques, diagnostic techniques (general aspects); iron; Iron - analysis; Liver - chemistry; Lung - chemistry; Magnetics; Medical sciences; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; Siderosis - etiology; Siderosis - metabolism; Spleen - chemistry; SQUID; thalassemia; Transfusion Reaction; Human; Hemoglobinopathy; Transfusion; Liver; Hepatic disease; Metabolic diseases; Hemopathy; Iron; Hemosiderosis; Blood; Genetic disease; Chemotherapy; Hemolytic anemia; Magnetometry; Treatment; β-Thalassemia; Non invasive method; Chelating agent; Digestive diseases; Complication; Metabolic storage
• ISSN: 0361-8609; 1096-8652
• DOI: 10.1002/(SICI)1096-8652(199904)60:4<289::AID-AJH7>3.0.CO;2-W

To investigate the applicability of noninvasive Superconducting Quantum Interference Device (SQUID) biomagnetic liver susceptometry and its limitations in thalassemic children, 23 patients with β‐thalassemia major and other iron loading anemias (age: 4–16 years) and 16 age‐related normal children were studied. Liver iron concentrations ranged from 600 to 11,000 μg/gliver for thalassemic patients and from 60 to 340 μg/gliver for normal patients. Measuring the respective organ volumes by sonography, liver and spleen iron stores, accounting for 80% of total body iron stores, were estimated. Nonliver contributions from the lung or intestine to the measured SQUID signals in the small‐sized patients were not observed. Moreover, livers in thalassemia were found to be enlarged by 18% per 1,000 μg/g (r = 0.75, P < 10−3). Serum ferritin values correlate significantly with iron stores (r = 0.64, P < 10−3), but predict iron stores only within large error intervals of 4,000 μg/gliver. Analyzing the experimental data from biomagnetometry and from related transfusion and chelation treatment data within the framework of a two‐compartment model, we were able to derive additional information on total body iron elimination and chelation therapy efficacy. The exponential decline of iron stores allows forecast of steady‐state conditions of the final iron load for a particular transfusion and chelation therapy regimen. Am. J. Hematol. 60:289–299, 1999. © 1999 Wiley‐Liss, Inc.