E3 Sickle Cell Anemia

Question 1

Which mutation causes sickle cell anemia?

Answer 1

HbS

Question 2

How do sickled cells affect blood flow?

Answer 2

The sickle cells adhere to the endothelium in the blood vessels blocking blood flow => ischemia

Question 3

What does nitric oxide have to do with sickle cell anemia?

Answer 3

Nitric oxide relaxes the blood vessels, helps RBCs get through the cell better [improves circulation]

Estrogen plays a role in the production of nitric oxide —> Women have higher nitric oxide levels

Men have increased morbidity —> Men and boys seem to be sicker bc of less production of nitric oxide

Question 4

Clinical Manifestations of SCA

Answer 4

Fatigue — anemia (sickled cells die quicker, cant replace fast enough)

Weakness — anemia (sickled cells die quicker, cant replace fast enough)

Pain — sickled blood cells blocking the vessels and decreasing blood flow to areas

Swelling — sickled blood cells blocking the vessels and decreasing blood flow to areas

Fever — infections; spleen makes and stores lymphocytes — when spleen is affected, affects their ability to fight off infections

Tachycardia — heart over-working trying to pump around the sickled cells

Murmurs — restricted blood flow can lead to heart problems

Enlargement of bones of the face and skull — bone marrow trying to make more RBCs because they are dying off faster

Question 5

Diagnostic