Thalassemia and Hemoglobinopathy

What is the amino acid substitution in patients with sickle cell anemia?

• adenine for thymine

• lysine for valine

• valine for glutamic acid

• glutamic acid for valine

Which of the following factors contributes to the pathophysiology of sickling?

• increased iron concentration

• hypochromia

• fava beans

• dehydration

Which of the following shows crystals like those seen here

• Hgb CC disease

• Hgb DD disease

• Hgb EE disease

• Hgb SC disease

The crystals seen here are associated with:

• Hgb CC disease

• Hgb SS disease

• Hgb SC disease

• Hgb EE disease

All but one of the following may be seen in the PB smear when a sickle cell crisis has occurred

• Target cells

• NRBCs

• Polychromasia

• Moderate elliptocytes

List two sets of lab data that can distinguish IDA from beta thalassemia trait.

• Serum iron and RBC

• Hgb and Hct

• WBC and RDW

• RBC indices and Platelets

What is the majority hemoglobin in beta thalassemia major

• Hgb A

• Hgb A2

• Hgb F

• Hgb H

Which of the following alpha thalassemia is inconsistent with life?

• Alpha thal trait

• Alpha thal minor

• Hgb H

• Hgb Bart's

Which of the following hemoglobins contains β₄ tetramers?

• Hgb Bart's

• Hgb Gower

• Hgb H

• Hgb Portland

Which of the following hemoglobins contains γ₄ tetramers?

• Hgb Bart's

• Hgb Gower

• Hgb H

• Hgb Portland