Commonest presentation of sickle cell anemia ?
Ans. is 'b' i.e., Bone pain Clinical manifestations of sickle cell anemia o 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. Vasoocclusive 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 - i) Painful bone crisis ii) Hand-foot syndrome Dactylitis of bones of hands/feet. iii) Autosplenectomy --> Predisposes to infection by encapsulated bacteria. iv) Acute chest syndrome v) Sequestration crisis Acute painful enlargement of spleen. vi) Aplastic crisis ----> due infection by parvovirus - B19 vii) CNS symptoms Seizure, stroke viii) Priapism and infarction of penis ix) Leg ulcers Remember o Sickle cell trait (heterozygous state) is almost always asymptomatic and the only significant manifestation is the renal concentrating defect presenting with isothenuria.
Priapism
Bone pain
Fever
Splenomegaly