Sickle Cell and Thalassemia

Question 1

what are acute clinical manifestations of sickle cell disease?

Answer 1

• vaso-occlusive and pain crises
• acute chest syndrome
• stroke
• sepsis
• chronic renal failure
• pulmonary hypertension
• iron overload

Question 2

hallmark of sickle cell disease?

Answer 2

Vaso-occlusive crisis (VOC)
* microvessel occlusion–> painful tissue hypoxia/infarction
* commonly affect long bones, back, chest, abdomen
* cosidered acute pain

Question 3

What often precipitates a vaso-occlusive crisis?

Answer 3

dehydration, cold temperatures, infection, exercise, stress- often no identifiable factor