Lecture 2020: Hemoglobinopathies

Question 1

What is the clinical severity, estimated hgb, and MCV for a homozygous sickle cell anemia patient?

Answer 1

Marked clinical severity
Hgb 6-9
MCV > 80

Question 2

What is the clinical severity, estimated hgb, and MCV for a patient with sickle-hgb C?

Answer 2

mild-moderate clinical severity
hgb 10-15
MCV 75-85

Question 3

When do babies with sickle cell anemia begin to manifest the disease? Why?

Answer 3

8-10 weeks of life.

They manifest at that time because there levels of hgb F begin to fall

Question 4

Folate dosage for pregnant woman with sickle cell?

Answer 4

5mg/day

Question 5

Basic management for pain crisis?

Answer 5

• Pain management with opiates
• hydration with D5-1/2NS
• Oxygen supplementation

Question 6

Cure for sickle cell anemia?

Answer 6

Stem cell transplant can be performed in children adolescents and represents only possible cure

Question 7

Name two 2nd line treatments for sickle cell anemia if hydroxyurea is ineffective.

Answer 7

L-glutamate (Endari)
Voxelator (Oxbryta)
Crizanlizumab (Adakveo)