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.
Priapism
Bone pain
Fever
Splenomegaly