title: Relationship Between Serum Hepcidin and Ferritin Levels in Patients With Thalassemia Major and Intermedia in Southern Iran.
abstract: BACKGROUND
  Hepcidin is a key regulator of iron absorption in humans. It is mainly affected by hypoxia and iron stores.
  
  
  OBJECTIVE
  The current study aimed to determine the correlation between serum hepcidin and ferritin levels in patients with Thalassemia Major (TM) and Thalassemia Intermedia (TI).
  
  
  METHODS
  The current cross-sectional study investigated 88 randomly selected patients with Thalassemia, 48 TM and 40 TI, registered at the Thalassemia Clinic of Shiraz University of Medical Sciences, a referral center for Thalassemia in Southern Iran in 2013. All patients with TI were receiving Hydroxyurea (HU) 10 - 15 mg/kg/day for at least 10 years. The serum hepcidin, ferritin levels, hemoglobin (Hb) and nucleated Red Blood Cell (RBC) of the two groups were measured.
  
  
  RESULTS
  No statistically significant correlation was observed between serum hepcidin and ferritin levels in any of the two groups of patients with TM (rs = 0.02, P = 0.892) or TI (rs = 0.055, P = 0.734). The median Interquartile Range (IQR) for serum hepcidin and ferritin levels were significantly higher in TM compared to TI group, (hepcidin: 87.6 (43.9) vs. 51.8 (23.4), P < 0.001; ferritin: 2208 (3761) vs. 465 (632), P < 0.001).
  
  
  CONCLUSIONS
  There was insignificant correlation between serum hepcidin and ferritin levels in the two groups of patients with TM and TI. It seems that regulation of hepcidin in patients with Thalassemia is more affected by erythropoeitic activity than iron stores. Also, hepcidin levels were significantly higher in patients with TM than TI, possibly due to higher erythropoeitic activity in TI. In TI, it seems that low dose HU increases Hb levels and leads to transfusion-independence, but it is not high enough to suppress bone marrow activity and ineffective erythropoiesis. Consequently, serum hepcidin level decreases.
authors.affiliation: Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, IR Iran.
authors.forename: Sezaneh
authors.initials: S
authors.lastname: Haghpanah
authors.affiliation: Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, IR Iran.
authors.forename: Masoomeh
authors.initials: M
authors.lastname: Esmaeilzadeh
authors.affiliation: Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, IR Iran ; Gastroentology Research Center, Shiraz University of Medical Sciences, Shiraz, IR Iran.
authors.forename: Naser
authors.initials: N
authors.lastname: Honar
authors.affiliation: Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, IR Iran.
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Hassani
authors.affiliation: Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, IR Iran.
authors.forename: Javad
authors.initials: J
authors.lastname: Dehbozorgian
authors.affiliation: Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, IR Iran.
authors.forename: Narges
authors.initials: N
authors.lastname: Rezaei
authors.affiliation: Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, IR Iran.
authors.forename: Maryam
authors.initials: M
authors.lastname: Abdollahi
authors.affiliation: Department of Library and Information Sciences, College of Humanities, Khouzestan Science and Research Branch, Islamic Azad University, Ahvaz, IR Iran.
authors.forename: Marzieh
authors.initials: M
authors.lastname: Bardestani
authors.affiliation: Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, IR Iran.
authors.forename: Sanaz
authors.initials: S
authors.lastname: Safaei
authors.affiliation: Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, IR Iran.
authors.forename: Mehran
authors.initials: M
authors.lastname: Karimi
keywords: Ferritin; Hepcidin; Hydroxyurea; Thalassemia Intermedia; Thalassemia Major