Hemolysis in Patients With Hereditary Spherocytosis (HS)

Overview

In the present study the investigators are going to explore the oxidative status of HS-RBC and its contribution to hemolysis

The oxidative status of cells, which is determined by the balance between pro-oxidants, such as the reactive oxygen species (ROS), and antioxidants, is a major regulator of cellular functions. Impaired balance between pro- and antioxidants causes oxidative stress which may result in oxidation of proteins, lipids and DNA with the final outcome of premature cell aging and apoptosis \[1,2\]. Oxidatively stressed red blood cell (RBC) have been observed in various congenital and acquired hemolytic anemias, including thalassemia, sickle cell anemia, congenital dyserythropoietic anemia, G6PD deficiency and paroxysmal nocturnal hemoglobinuria (PNH) as well as in myelodysplastic syndrome (MDS). Although the primary etiology is different in these anemias, oxidative stress mediates several of their pathologies, mainly hemolysis \[3\]. Hereditary Spherocytosis (HS) is a genetic disorder of the RBC skeleton with primary deficiency in spectrin, ankyrin-1, band 3 or protein 4.2 associated with chronic hemolytic anemia \[4\]. Secondary protein deficiencies resulting from oxidative stress are often observed and may be involved in the clinical manifestations of the disease \[5\].

Conditions

Interventions

Eligibility

Locations (1)

Outcomes

Central Contacts