Krafts Anemia 2

Question 1

Best test for hemoglobinopathy?

Answer 1

Hgb electrophoresis

Question 2

Most important hemoglobinopathy?

Answer 2

Sickle cell

Question 3

Genetic cause of sickle cell?

Answer 3

point mutation in Beta chain gene

Question 4

“Post-splenectomy blood picture” of sickle cell?

Answer 4

• nucleated RBCs
• targets
• Howell-Jolly bodies
• Pappenheimer bodies
• incr. platelets

Question 5

Bugs to vaccinate against in sickle cell?

Answer 5

encapsulated:

S. pneumo

H. influenzae

Question 6

4 thing you must know about Thalassemia?

Answer 6

1. QUANTITATIVE defect in hgb
2. can’t make enough alpha or beta chains
3. variable severity
4. hypochromatic, microcytic anemia with incr. RBC and targets

Question 7

What’s worse, alpha thalasemia or beta?

Answer 7

alpha: if no alpha chains, can’t make hgb

no beta can still have some alpha-delta (HgbA2)

Question 8

Morphology of Thalasemia:

Answer 8

• hypochromic, microcytic anemia
• target cells
• basophilic stippling
• depending on severity: may have minimal anisocytosis/poikilocytosis or marked of both

Question 9

a-thalasemia common demographics:

Answer 9

asians, blacks

Question 10

b-thalasemia common demographics:

Answer 10

mediterranian and blacks

Question 11

How does G6PD deficiency cause anemia?

Answer 11

no glutathione (NADP can’t get reduced to NADPH) to clear radical peroxides –> cell lysis

Question 12

Bite cells are characteristic of?

Answer 12

G6PD deficiency

*removal of Heinz bodies in Spleen

Question 13

Whats a Heinz body?

Answer 13

clump of globin stuck to RBC membrane

Question 14

Triggers of G6PD def?

Answer 14

• fava beans

- drugs (abx, asa)

Question 15

Whats a big red flag for microangiopathic hemolytic anemia?

Answer 15

Schistocytes (deformed RBCs)

-physical trauma to RBCs

***better find out why!!!

Question 16

Triangle cells classic for:

Answer 16

MAHA (microangiopathic hemolytic anemia)

Question 17

Why is hgb normal at first in acute blood loss anemia?

Answer 17

hgb is a ratio and pt will be volume depleted

-must check after fluid resuscitation

***Reticulocytes show up after 2-3 days

Question 18

Does anemia of chronic disease have reticulocytes?

Answer 18

NO

Question 19

Things you must know about anemia of chronic disease?

Answer 19

1. infx, inflammation, malignancy
2. Fe metabolism disturbed (incr. hepcidin)
3. normocytic, normochromatic anemia
4. usually mild anemia

Question 20

Cause of anemia of renal disease?

Answer 20

lack of EPO

Question 21

What are echinocytes and when would you see them?

Answer 21

“spiny” cells

seen in anemia of renal disease

Question 22

Things to know about anemia of liver disease:

Answer 22

common (3/4 of pts with liver disease are anemic)

many causes

“uncomplicated” cases rare

acanthocytes, targets

Question 23

Things to know about aplastic anemia?

Answer 23

pancytopenia

empty marrow

most are idiopathic