Alpha‐thalassaemia promotes frequent vaso‐occlusive crises in children with sickle cell anaemia through haemorheological changes

Abstract

Our results demonstrate that alpha-thalassaemia is associated with higher risk for VOC events in children with SCA, which may be due in part to its effects on RBC deformability and aggregation.

Rheological properties of sickle erythrocytes in patients with sickle‐cell anemia: The effect of hydroxyurea, fetal hemoglobin, and α‐thalassemia

“…[19][20][21] The rheology of blood, in deletes was too small), deformability of sickle RBCs from subjects with coexistent α-thalassemia is better than that from non-thalassemic patients with SCA as shown in this and other studies. 20,21,[26][27][28] Subjects with SCA and α-thalassemia have less (ISCs) and dense cells in their peripheral blood than non-thalassemic patients. Moreover, the effect of α-thalassemia in response to HU therapy was recently described in the Multicenter Study of Hydroxyurea (MSH) cohort.…”

Sickle cell disease: Classification of clinical complications and approaches to preventive and therapeutic management

“…The increased Hb level associated with α gene deletion, however, presumably increases the blood viscosity, which is often accompanied by increased frequency of painful crises [14,15] and vaso-occlusive episodes such as avascular necrosis (AVN) [16,17]. Recently, Renoux et al [18] observed increased frequency of crises in children with alpha-thalassemia but it was not explained by increased blood viscosity.…”

“…The number of patients studied, however, was relatively small and the diagnosis of avascular necrosis was not ruled out [18]. The effect of α gene deletion on the clinical picture is best illustrated in SCA with two α gene deletions.…”

Measuring success: utility of biomarkers in sickle cell disease clinical trials and care

“…First, hypertransfusion has been associated with neurological symptoms in SCD. 47 Second, patients with HbSC disease or HbSS in combination with a-thalassemia also have a high rate of VOCs, 48,49 despite lower degrees of anemia and hemolysis. 5 In these patients, hyperviscosity has been implicated as the predominant pathway to vaso-occlusion.…”