Study of alpha hemoglobin stabilizing protein expression in patients with β thalassemia and sickle cell anemia and its impact on clinical severity

• Published in: Blood cells, molecules, & diseases molecules, & diseases, 2015-12, Vol.55 (4), p.358-362
• Main Authors: Mahmoud, Hanan Mohamed, Shoeib, Ahmed Al-Saiid Hamed, Abd El Ghany, Shereen Mohamed, Reda, Marwa Mohamed, Ragab, Iman Ahmed
• Format: Article
• Language: English
• Subjects: AHSP; Anemia, Sickle Cell - blood; Anemia, Sickle Cell - diagnosis; Anemia, Sickle Cell - genetics; Anemia, Sickle Cell - therapy; beta-Thalassemia - blood; beta-Thalassemia - diagnosis; beta-Thalassemia - genetics; beta-Thalassemia - therapy; Blood Proteins - genetics; Child; Child, Preschool; Clinical severity; Cross-Sectional Studies; Female; Gene Expression Regulation; Humans; Infant; Male; Molecular Chaperones - genetics; NTDT; Severity of Illness Index; Sickle cell anemia; β-Thalassemia
• ISSN: 1079-9796; 1096-0961
• DOI: 10.1016/j.bcmd.2015.07.016

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