Intro + Hemoglobinopathies and Thals

Question 1

What is HbF

Answer 1

alpha gamma

Question 2

What is HbA

Answer 2

alpha beta

Question 3

What is HbA2

Answer 3

alpha delta

Question 4

What is HbH

Answer 4

beta 4

Question 5

What is Hb Barts

Answer 5

gamma 4

Question 6

Difference between thalassemia and a hemoglobinopathy

Answer 6

Thal – Quantity Problem

Hemoglobinopathy – abnormal structure/fxn

Question 7

What is alpha0-thalassemia minor. Who gets it?

Answer 7

Two alpha genes deleted/altered
a-thal 1 = __/aa –> South East Asia
a-thal 2 = a_/a_ –> African American

Question 8

Cause of beta thal intramedullary hemolysis

Answer 8

alpha4 adheres to RBC precursor membrane

Question 9

Problem in methemoglobinemia

Answer 9

heme contains Fe3+ Iron instead of 2+ – can’t hold O2 as well

Question 10

Triggers of methemoglobinemia

Answer 10

Cytochrome b5 reductase deficiency

Lidocaine

Question 11

What is the amino acid change that triggers sickle cell? Hemoglobin C?

Answer 11

6th Codon on Beta Globin Chain
Glutamic Acid –> Valine
Glutamic Acid –> Lysine

Question 12

Aside from the whole HbS polymers thing, how else does Sickle Cell mess with your CV system

Answer 12

hemolysis leads to arginase and free Hb release

Free Hb consumes NO