With the global scope of sickle-cell disease, knowledge of the countless clinical
presentations and treatment of this disorder need to be familiar to generalists …

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 …

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

Sickle cell disease is a common and life-threatening haematological disorder that affects
millions of people worldwide. Abnormal sickle-shaped erythrocytes disrupt blood flow in …

The discovery of the molecular basis of sickle cell disease was an important landmark in
molecular medicine. The modern tools of molecular and cellular biology have refined our …

In sickle-cell disease, a point mutation in the β-globin chain causes haemoglobin to
polymerise within erythrocytes during deoxygenation, altering red blood cell rheology and …

Context New therapies have evolved from our improved understanding of the biology of
sickle cell disease (SCD) and the availability of a useful transgenic animal model. Several …

Sickle cell disease (SCD) is a heterogeneous disorder, with clinical manifestations including
chronic haemolysis, an increased susceptibility to infections and vaso-occlusive …

In sickle cell disease, a single base pair substitution in the gene encoding the β-globin chain
of the haemoglobin molecule gives rise to a surprisingly broad spectrum of …

Sickle cell disease (SCD) is a complex, clinically heterogeneous disorder affecting
approximately 100,000 individuals in the United States and millions worldwide. The disease …