title: Study of alpha hemoglobin stabilizing protein expression in patients with β thalassemia and sickle cell anemia and its impact on clinical severity.
abstract: The α hemoglobin stabilizing protein (AHSP) binds α-Hb and prevents its precipitation limiting free α-Hb toxicities. Our aim was to study AHSP expression in β thalassemia syndromes in relation to their clinical severity and to compare it with its level in sickle cell anemia. We compared patients with β-thalassemia (n=37) (β-thalassemia major (BTM) (n=19) and β-thalassemia intermedia (BTI) (n=18)) with 12 patients with sickle cell anemia as regards clinical severity, age at presentation, transfusion dependency, mean pre-transfusion hemoglobin level, use of hydroxyurea and AHSP expression by real time quantitative PCR. Median (and IQR) AHSP expression was significantly higher in patients with sickle cell anemia 2275 (3898) compared to thalassemia 283 (718), P=0.001, with no significant difference between BTM and BTI (P=0.346). It was also significantly higher in non-transfusion dependent patients with β thalassemia (NTDT) compared to transfusion dependent ones (P=0.019), and in patients on hydroxyurea therapy (P<0.001). However, there was no significant difference in its level according to clinical severity score (P=0.946) or splenectomy status (P=0.145).
  
  
  CONCLUSIONS
  AHSP expression was higher in patients with sickle cell anemia versus thalassemia, with no significant difference between BTM and BTI. Expression was higher in patients with NTDT and on hydroxyurea therapy.
authors.affiliation: Clinical Pathology Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Hanan Mohamed
authors.initials: HM
authors.lastname: Mahmoud
authors.affiliation: Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Ahmed Al-Saiid Hamed
authors.initials: AA
authors.lastname: Shoeib
authors.affiliation: Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Shereen Mohamed
authors.initials: SM
authors.lastname: Abd El Ghany
authors.affiliation: Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Marwa Mohamed
authors.initials: MM
authors.lastname: Reda
authors.affiliation: Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt. Electronic address: hragab68@hotmail.com.
authors.forename: Iman Ahmed
authors.initials: IA
authors.lastname: Ragab
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy; D001798:Blood Proteins / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D005786:Gene Expression Regulation; D006801:Humans; D007223:Infant; D008297:Male; D018832:Molecular Chaperones / Q000235:genetics*; D012720:Severity of Illness Index; D017086:beta-Thalassemia / Q000097:blood / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy
chemical_list: C468768:AHSP protein, human; D001798:Blood Proteins; D018832:Molecular Chaperones
keywords: AHSP; Clinical severity; NTDT; Sickle cell anemia; β-Thalassemia