Conflict of Interest: Dr. Wood, Dr. Coates, and Dr. Harmatz have research funding from Novartis and had received speakers honoraria from Novartis and Apotex.
Patterns of hepatic iron distribution in patients with chronically transfused thalassemia and sickle cell disease†
Article first published online: 18 MAY 2009
Copyright © 2009 Wiley-Liss, Inc.
American Journal of Hematology
Volume 84, Issue 8, pages 480–483, August 2009
How to Cite
Ghugre, N. R., Gonzalez-Gomez, I., Butensky, E., Noetzli, L., Fischer, R., Williams, R., Harmatz, P., Coates, T. D. and Wood, J. C. (2009), Patterns of hepatic iron distribution in patients with chronically transfused thalassemia and sickle cell disease. Am. J. Hematol., 84: 480–483. doi: 10.1002/ajh.21456
- Issue published online: 24 JUL 2009
- Article first published online: 18 MAY 2009
- Accepted manuscript online: 18 MAY 2009 12:00AM EST
- Manuscript Accepted: 13 MAY 2009
- Manuscript Revised: 12 MAY 2009
- Manuscript Received: 20 OCT 2008
- National Institutes of Health (General Clinical Research Center). Grant Number: RR00043-43
- Pediatric Clinical Research Center at the Children's Hospital & Research Center, Oakland. Grant Number: M01 RR01271
- Center for Disease Control (Thalassemia Center). Grant Number: U27/CCU922106
- Department of Pediatrics at Children's Hospital Los Angeles (CHLA)
- National Heart Lung and Blood Institute of the National Institutes of Health. Grant Number: 1 R01 HL75592-01A1
- National Institute for Diabetes, Digestion, and Kidney Disease. Grant Number: R01 DK057778
- Saban Research Institute at CHLA
Patients with sickle cell disease (SCD) appear to be at lower risk of endocrinopathies and cardiac dysfunction than those with thalassemia major (TM). Circulating redox active iron is lower in these patients, possibly due to increased systemic inflammation and circulating cytokines. Hepcidin synthesis is upregulated during chronic inflammation, reducing intestinal iron absorption and promoting retention of iron in the reticuloendothelial cells. Hence, we hypothesized that livers of patients with SCD would exhibit greater iron deposition in sinusoidal spaces relative to hepatocytes and less in portal tracts when compared to patients with TM. To test this hypothesis, iron scoring analysis was performed on 70 clinically indicated liver biopsy specimens from children and young adults with the two syndromes. Sinusoidal scores were lower in around 1 of 4 patients with TM but the relative iron loading in hepatocytes, and portal tracts was identical in both diseases. Sinusoidal iron burdens saturated at low hepatic iron concentration (HIC) while hepatocyte and portal iron depots increased proportionally to HIC. Liver fibrosis was increased in patients with TM regardless of their chronic hepatitis status. Overall, liver iron distribution was relatively insensitive to differences in disease type and to the presence or absence of hepatitis. Am. J. Hematol., 2009. © 2009 Wiley-Liss, Inc.