Hemoglobinopathies

Front 1

Name the 3 major Hemoglobins that are present in mature red blood cells

Back 1

Hemoglobin A(98%), A2, and F

Front 2

How do the various Hemoglobins differ and how are they similar?

Back 2

They all share globin chain alpha but differ in the second globin chain.

Front 3

What feature allows Hb F to cross the placenta?

Back 3

It's affinity for oxygen.

Front 4

When does rbc production begin in the embryo?

Back 4

By day 19 in the yolk sac.

Front 5

What are the forms of embryonic Hb? & What how do they fxn after birth?

Back 5

Gower 1, Gower 2, and Portland. These Hb's are of no clinical importance after birth.

Front 6

These are defined as mutations that affect the production of normal Hb chains

Back 6

Thalessemia syndromes

Front 7

What are 4 major genetic abnormalities that can occur in globin synthesis?

Back 7

1. Decreased solubility
2. Oxygen Transport (w/ high/low O2 affinity)
3. Oxygen reduction status
4. Monomeric state

Front 8

Why do most of the hemoglobinopathies affect the beta chain?

Back 8

Because there are 4 copies of the alpha chain.

Front 9

Name the 4 types of hemoglobinopathies studied?

Back 9

1) sickle cell syndromes
2) unstable Hb's
3) Hb's w/ altered O2 affinity
4) Hb M

Front 10

What are some promoters of rbc cell sickling?

Back 10

hypoxia, low pH, 2,3DPG, reduced rbc H2O, and fever

Front 11

Name two tests for sickle cell syndrome?

Back 11

1) sickle prep test w/ dithionate or metabisulfate

2) Hb electrophoresis

Front 12

This is the dominant cause of morbidity and mortality in HbSS (sickle cell anemia)

Back 12

vasocclusive crisis

Front 13

What is splenic sequestration crisis?

Back 13

the spleen enlarges in kids and Hb drops; the child bleeds into the spleen

Front 14

Aplastic crisis

Back 14

Is due to Parvovirus B19 or secondary folic acid deficiency; occurs when rbc production turns off

Front 15

Name two tests for sickle cell syndrome?

Back 15

1) sickle prep test w/ dithionate or metabisulfate

2) Hb electrophoresis