Microcytic Anemias

Question 1

An MCV < 80 ?

Answer 1

Microcytic anemia

Question 2

What is are the RBC’s smaller?

Answer 2

They undergo an extra division to maintain hemoglobin concentration

Question 3

What are the four main microcytic anemias?

Answer 3

1. iron deficiency
2. Anemia of chronic disease
3. Sideroblastic anemia
4. Thalessemia

Question 4

What are the lab tests for iron?

Answer 4

1. serum iron
2. TIBC transferritin
3. % sat of transferritin
4. Ferritin

Question 5

what is heme composed of?

Answer 5

iron and protoporphyrin

Question 6

What is the most common nutritional deficiency worldwide?

Answer 6

iron, 33%

Question 7

How is iron absorbed?

Answer 7

Duodenum. Entry into enterocytes (DMT1). Exit through transportin to transferritin.

Question 8

What are the stages of iron deficiency?

Answer 8

1. Storage depletion (decreased ferritin)
2. Serum iron is depleted (serum iron and % sat)
3. Normocytic anemia
4. microcytic, hypochromic anemia

Question 9

What are the clinical features of iron deficiency anemia?

Answer 9

anemia, koilonychia (spoon nails), pica (goat)
decreased ferritin/increased TIBC (always reciprocal)
decreased serum iron
decreased % sat
increased FEP

Question 10

What syndrome presents with esophageal web and atrophic glossitis?

Answer 10

Plummer Vinson Syndrome (iron defic)

Question 11

What anemia is associated with chronic inflammation?

Answer 11

Anemia of Chronic disease

Question 12

What acute phase protein sequesters iron and decreases EPO?

Answer 12

hepcidin

Question 13

What condition has:
increased ferritin/decreased TIBC
decreased serum iron
decreased % sat
increased FEP?

Answer 13

Anemia of Chronic disease

Question 14

What anemia causes an iron overloaded state?

Answer 14

sideroblastic

Question 15

Defective protoporphyrin synthesis causes?

Answer 15

sideroblastic anemia

Question 16

What congenital defect affects heme synthesis?

Answer 16

ALAS defect (rate limitning
succ CoA--> aminolevulinic acid (ALA)

Question 17

Vit B6 is a co factor for?

Answer 17

ALAS (aminolevulinic acid synthase)

Question 18

What step or heme synthesis occurs in mitochondria?

Answer 18

ferrochetalase. Iron laden mito ring the nucleus (sideroblasts)

Question 19

What condition has:
increased ferritin/ decreased TIBC
increased serum iron
decreased % sat

Answer 19

sideroblastic anemia

Question 20

Decreased synthesis of globin chains?

Answer 20

thalasemia

Question 21

4 alleles on Chr 16

Answer 21

alpha chains

Question 22

2 allles on chr 11

Answer 22

beta chains

Question 23

Cis 2 gene deletion of alpha alleles is common where?

Answer 23

Asia, more severe when passed on

Question 24

Trans 2 gene alpha allele deletion is common where?

Answer 24

Africa

Question 25

What is HBH

Answer 25

tetramers of beta chains

Question 26

what is Hb Barts?

Answer 26

no alpha chains–> hydrops fetalis

4 alleles lost

Question 27

What causes sever alpha thalassemia?

Answer 27

3 gene deletion

Question 28

what is Bnull and B+

Answer 28

minor thalassemia

Question 29

What causes major thalassemia

Answer 29

Bnull/Bnull

Question 30

Of what condition are target cells seen?

Answer 30

minor thalassemia

Question 31

in what condition can EBV cause an aplastic crisis?

Answer 31

Major thalassemia

Question 32

What are the clinical features of Major thalassemia?

Answer 32

1. A2/A2 tetramers (no B chains)
2. extravascular hemolysis
3. Massive erythroid hyperplasia (crew cut skull, chipmunk face)
4. extramedullary hematopoesis
   nucleated RBC’s
   target cells