Heme1c

Question 1

What are the 3 forms of embryonic hb?

Answer 1

a. Gower 1: zeta2 epsilon2
b. Portland: zeta2 gamma2
c. Gower 2: alpha2 epsilon3

Question 2

What are the chain components in fetal hb?

Answer 2

alpha2 gamma2

Question 3

What are the components, names and percentages in adult hb?

Answer 3

alpha2 delta2, A2, 1.5-3.5%

alpha2 beta2, A, >95%

Question 4

When does the beta gamma switch happen?

Answer 4

3-4 months gestation is when it begins.

Question 5

At birth, what percent of Hb is fetal?

Answer 5

~80-90%

Question 6

What causes a left shift in the O2 dissociation curve?

Answer 6

up: pH
down: temp, pCO2, 2,3-DPG

hb type: fetal

Question 7

What causes a right shift in the O2 dissociation curve?

Answer 7

down: pH
up: temp, pCO2, 2,3-DPG

hb type: adult

Question 8

Tell me about inheritance and clinical manifestations of G6P deficiency.

Answer 8

X-linked, also many drugs cause it.

Asymptomatic, neonatal hyperbilirubinemia, chronic hemolysis, intermittent hemolysis (stressors,- fava beans).

Late complications: diabetes, renal failure

Question 9

What do you see on a smear for G6P deficiency?

Answer 9

Heinz bodies and “bites” take out of the RBC.

Question 10

How do you Dx G6P deficiency?

Answer 10

CBC retic count
Smear:  Heinz bodies, bite cells
LFTs:  increased bilirubin
Haptoglobin
Enzyme assay
Genetic testing

Question 11

What is the difference between Howell-Jolly and Heinz bodies?

Answer 11

Howell-Jolly: Nuclear remanants, found in damaged spleen (trauma, radiation, etc.) and megaloplastic anemia.

Heinz: gummed up globlin chains in thalassemias.

Question 12

What factors does PT test for?

Answer 12

I, II,V, VII, and X

Question 13

What factors does PTT test for?

Answer 13

Slides: II, V, VIII, IX, X, and XI

First Aid: All except VII and XIII

Question 14

A deficiency in what leads to Hemophilia A? B? What do they do to the PTT?

Answer 14

A: VIII
B: IX
PTT: both increase the PTT