Commonest presentation of sickle cell anemia –

Clinical manifestations of sickle cell anemia The clinical manifestations of sickle cell anemia are - 1. Chronic hemolysis Irreversible sickle cells have rigid, nondeformable cell membranes that lead to difficulty in negotiating the splenic sinusoids, sequestration, and rapid phagocytosis. This results in extra-vascular hemolysis. Some intravascular hemolysis may also occur because of increased mechanical fragility of severely damaged cells. 2. Vasoocclnsive symptoms Reversible sickle cells express higher than normal levels of adhesion molecules and are abnormally sticky that is responsible for occlusion of microcirculation especially of bones, lungs, spleen and penis. Vasoocclusive symptoms are the most common manifestations of sickle cell anemia. They commonly manifest as - Painful bone crisis Hand-foot syndrome → Dactylitis of bones of hands/feet. Autosplenectomy →Predisposes to infection by encapsulated bacteria. Acute chest syndrome Sequestration crisis → Acute painful enlargement of spleen. Aplastic crisis → due to infection by parvovirus - B19 CNS symptoms → Seizure, stroke Priapism and infarction of penis Leg ulcers Remember Sickle cell trait (heterozygous state) is almost always asymptomatic and the only significant manifestation is the renal concentrating defect presenting with isosthenuria.