Managing Acute Complications of Sickle Cell Disease

Sickle cell anaemia (SCA) is an autosomal recessive haemoglobinopathy caused by a single gene mutation. In this condition, hydrophilic glutamic acid is replaced by hydrophobic valine at the sixth amino acid position of the beta chain, forming haemoglobin S (HbS). HbS polymerises under deoxygenated conditions, deforming the erythrocyte membrane. The tissue and organ ischaemia that occurs in SCA leads to both acute and chronic complications in affected patients. Acute complications can result in high mortality and morbidity if not managed effectively. Simple transfusion and erythrocyte exchange (RBC exchange) play important roles in treatment. Today, RBC exchange can be performed safely with the help of new-generation apheresis devices. However, certain limitations persist, including the need for experienced personnel in apheresis procedures, the frequent requirement for a central venous catheter, and the risk of complications related to blood product use. This chapter will discuss medical therapies for managing acute SCA complications, along with simple transfusion and RBC exchange therapies and their indications.