Hemolysis is a fundamental feature of sickle cell anemia that contributes to its
pathophysiology and phenotypic variability. Decompartmentalized hemoglobin, arginase 1 …

Sickle-cell disease is one of the most common severe monogenic disorders in the world.
Haemoglobin polymerisation, leading to erythrocyte rigidity and vaso-occlusion, is central to …

A randomized, controlled clinical trial established the efficacy and safety of short‐term use of
hydroxyurea in adult sickle cell anemia. To examine the risks and benefits of long‐term …

Sickle cell disease is characterized by recurrent episodes of ischemia-reperfusion injury to
multiple vital organ systems and a chronic hemolytic anemia, both contributing to …

Sickle cell disease (SCD), caused by a mutation in the β-globin gene HBB, is widely
distributed in malaria endemic regions. Cardiopulmonary complications are major causes of …

Sickle cell disease is an autosomal recessive, multisystem disorder, characterised by
chronic haemolytic anaemia, painful episodes of vaso-occlusion, progressive organ failure …

Abstract The “hyperhemolytic paradigm”(HHP) posits that hemolysis in sickle disease
sequentially and causally establishes increased cell‐free plasma Hb, consumption of NO, a …

Aims Cardiac involvement is common in sickle cell disease (SCD). Studies are needed to
establish haematological determinants of this involvement and prognostic markers. The aim …

Hemolysis is a key feature of sickle cell anemia (HbSS). Direct quantitation of hemolysis
could be used as an objective outcome in clinical trials of new therapeutics for HbSS and …

In the US, mortality in sickle cell disease (SCD) increases after age 18‐20 years. Biomarkers
of mortality risk can identify patients who need intensive follow‐up and early or novel …