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

Abstract

UNLABELLED:

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).

CONCLUSION:

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: Hanan Mohamed Mahmoud, Ahmed Al-Saiid Hamed Shoeib, Shereen Mohamed Abd El Ghany, Marwa Mohamed Reda, Iman Ahmed Ragab
• Journal: Blood cells, molecules & diseases (Blood Cells Mol Dis) Vol. 55 Issue 4 Pg. 358-62 (Dec 2015) ISSN: 1096-0961 [Electronic] United States
• PMID: 26460260 (Publication Type: Journal Article)
• Copyright: Copyright © 2015 Elsevier Inc. All rights reserved.

Chemical References

• AHSP protein, human
• Blood Proteins
• Molecular Chaperones

Topics

• Anemia, Sickle Cell (blood, diagnosis, genetics, therapy)
• Blood Proteins (genetics)
• Child
• Child, Preschool
• Cross-Sectional Studies
• Female
• Gene Expression Regulation
• Humans
• Infant
• Male
• Molecular Chaperones (genetics)
• Severity of Illness Index
• beta-Thalassemia (blood, diagnosis, genetics, therapy)