Elevated liver iron concentration is a marker of increased morbidity in patients with β thalassemia intermedia

Khaled M. Musallam, Maria Domenica Cappellini, John C. Wood, Irene Motta, Giovanna Graziadei, Hani Tamim, Ali T. Taher

Research output: Contribution to journalArticlepeer-review

Abstract

Background Patients with β thalassemia intermedia can have substantial iron overload, irrespectively of their transfusion status, secondary to increased intestinal iron absorption. This study evaluates whether iron overload in patients with β thalassemia intermedia is associated with morbidity. Design and Methods This was a cross-sectional study of 168 patients with β thalassemia intermedia treated at two centers in Lebanon and Italy. Data on demographics, splenectomy status, transfusion status, and presence of co-morbidities were retrieved. Laboratory values of serum ferritin, fetal and total hemoglobin levels, as well as platelet and nucleated red blood cell counts were also obtained. Iron burden was determined directly by measuring liver iron concentration using magnetic resonance imaging. Patients were subdivided according to transfusion and splenectomy status into groups with phenotypes of different severity. Results The mean age of the patients was 35.2±12.6 years and 42.9% of them were male. The mean liver iron concentration was 8.4±6.7 mg Fe/g dry weight. On multivariate logistic regression analysis, after adjusting for age, gender, splenectomy status, transfusion status, and laboratory indices, an increase in 1 mg Fe/g dry weight liver iron concentration was independently and significantly associated with higher odds of thrombosis, pulmonary hypertension, hypothyroidism, osteoporosis, and hypogonadism. A iver iron concentration of at least 7 and at least 6 mg Fe/g dry weight were the best thresholds for discriminating the presence and absence of vascular and endocrine/bone morbidities, respectively (area under the receiver-operating characteristic curve: 0.72, P

Original languageEnglish
Pages (from-to)1605-1612
Number of pages8
JournalHaematologica
Volume96
Issue number11
DOIs
Publication statusPublished - Nov 1 2011

Keywords

  • Endocrine disease
  • Iron overload
  • Liver iron concentration
  • Osteoporosis
  • Thalassemia intermedia
  • Vascular disease

ASJC Scopus subject areas

  • Hematology

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