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Flashcards in Hematology Deck (207)
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1

What is a sideroblast?
A ringed sideroblast?

RBC with excessive iron in its mitochondria.
Normal to see these in the bone marrow.
Ringed sideroblasts = never normal. Have a ring of iron around the nucleus.

2

Clinical conditions in which you might see ringed sideroblasts (6):

Lead poisoning
Heme metabolism d/o
EtOH
Drugs
Genetic disorders
Myelodysplastic syndrome

3

Four scenarios in which you might see target cells:

THAL:
Thalassemias
HbC disease
Asplenia
Liver disease

4

Two pathologic RBC forms you might see in someone with liver disease:

Target cells
Acanthocytes

5

What is physiologic anemia of pregnancy?

Increase in maternal plasma volume -> dilution of iron in the serum.

6

Iron deficiency anemia is a _________ __________ anemia.

Microcytic
Hypochromic

7

This type of anemia may manifest as Plummer-Vinson syndrome:
The famous triad:

Iron deficiency
Fe-deficiency
Esophageal webs
Atrophic glossitis

8

How many copies of the gene for a-globin do you have?

Four.

9

Which thalassemia is more prevalant in people of Mediterranean descent?
In Asian people?
African people?

b-thal = Mediterranean
a-thal = Asian, African

10

What is Hb Barts?

People make no a-globin.
In the fetus, so...
Gamma-globin makes a tetramer = Hb Barts

11

What is HbH disease?

HbH disease = 3-gene deletion of a-globin.
Make only a little a-globin
The extra b-globin makes tetramers = HbH

12

Four gamma-globin chains glued together, what am I?

Big trouble, you're not making alpha-globin chains in utero.
Hb Barts -> hydrops fetalis.

13

Four beta-globin chains glued together, what am I? What disease is this associated with?

HbH -> HbH disease.
A type of alpha-thalassemia.

14

How many genes for b-globin do you have?

Two.

15

When might you see target cells?

THAL
Thalassemia
HbC disease
Asplenia
Liver disease

16

How do you confirm the dx of b-thalassemia minor?

Will see an increase in HbA2.
HbA2 = a-globin + d-globin

17

Which thalassemia commonly puts people at risk for hemochromatosis?

b-thalassemia major (homozygous)
Often require blood transfusions.

18

Two skeletal abnormalities seen in b-thalassemia major:

Marrow expansion ->
"Crew cut" appearance on X-ray
"Chipmunk" facies

19

What is the structure of HbF?

a2g2

20

The macrocytic anemias can be divided into these two functional distinctions:
What is the difference between them?

Megaloblastic = trouble synthesizing DNA
Non-megaloblastic = unimpaired DNA synth.

21

Three causes of megaloblastic anemia:

Folate def.
B12 def.
Orotic aciduria

22

Common finding on blood smear in megaloblastic anemia (other than big-ass RBC):

Hypersegmented neutrophils

23

Anemia in which methylmalonic acid is normal:
Anemia in which methylmalonic acid is elevated:

Anemia 2/2 folate deficiency
Anemia 2/2 B12 deficiency

24

Four common causes of B12 deficiency:

Malabsorption
Poor intake
Pernicious anemia
PPIs
(Worms)

25

Which of the macrocytic anemias would you expect to see neurologic sx with?

B12 deficiency anemia

26

This macrocytic anemia is incurable by folate / B12 administration:

Anemia 2/2 orotic aciduria

27

What is the treatment for orotic aciduria?

Uridine monophosphate

28

In B12 and folate deficiency anemias, what is the homocysteine level like?

Increased.

29

What is the protein responsible for binding ferroportin in anemia of chronic disease?

Hepcidin

30

What kind of anemia is anemia of chronic disease?

Normocytic
May progress to microcytic, hypochromic