SICKLE CELL DISEASE   

 
 

Symptoms: Bone pain; weakness; jaundice occasionally
Special signs: Anemia; bone pain crisis, hypersplenism or auto infarction of spleen, cachexia, hepatomegaly. May also have respiratory tract infection which may cause sputum production, cough as a result of pneumonia.
Special investigations: Sickling +; HbS electrophoresis; Mean corpuscular hemoglobin concentration (MCHC) is increased.

SHORT DESCRIPTION:
This is a disease cause by defective HbS gene which then leads red cells to sickle. With the sickling comes the elimination of the red cells by the reticuloendothelial cells leading to hemolytic anemia and jaundice. The disease may be complicated by malaria, respiratory tract infection in addition to the common bone pain crises caused by blockage of blood vessels supplying the bones, autoinfarction of spleen and many other end organs leading to a plethora of symptoms and signs. MCHC is markedly increased and facilitates sickling. Other crises includes acute sequestration crises and hyperhemolytic crisis.

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