sickle cell anaemia (2)

Hematology

Sickle cell disease (SCD) is a hereditary disorder characterized by the production of abnormal hemoglobin (HbS), which causes red blood cells to adopt a sickle shape under low oxygen tension. The document outlines the classification of SCD, including sickle cell anemia (homozygous SS), sickle cell trait (heterozygous AS), and other compound heterozygous conditions like Hb SC and Hb S-beta-thalassemia. It delves into the molecular basis of sickling, the clinical manifestations such as various types of crises (vaso-occlusive, hyperhemolytic, aplastic, sequestration), susceptibility to infections, and potential organ damage. Diagnostic methods including peripheral smear analysis, bone marrow examination, sickling tests, and hemoglobin electrophoresis are also discussed.