Flashcards in Hematology Deck (207)
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.
Clinical conditions in which you might see ringed sideroblasts (6):
Heme metabolism d/o
Four scenarios in which you might see target cells:
Two pathologic RBC forms you might see in someone with liver disease:
What is physiologic anemia of pregnancy?
Increase in maternal plasma volume -> dilution of iron in the serum.
Iron deficiency anemia is a _________ __________ anemia.
This type of anemia may manifest as Plummer-Vinson syndrome:
The famous triad:
How many copies of the gene for a-globin do you have?
Which thalassemia is more prevalant in people of Mediterranean descent?
In Asian people?
b-thal = Mediterranean
a-thal = Asian, African
What is Hb Barts?
People make no a-globin.
In the fetus, so...
Gamma-globin makes a tetramer = Hb Barts
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
Four gamma-globin chains glued together, what am I?
Big trouble, you're not making alpha-globin chains in utero.
Hb Barts -> hydrops fetalis.
Four beta-globin chains glued together, what am I? What disease is this associated with?
HbH -> HbH disease.
A type of alpha-thalassemia.
How many genes for b-globin do you have?
When might you see target cells?
How do you confirm the dx of b-thalassemia minor?
Will see an increase in HbA2.
HbA2 = a-globin + d-globin
Which thalassemia commonly puts people at risk for hemochromatosis?
b-thalassemia major (homozygous)
Often require blood transfusions.
Two skeletal abnormalities seen in b-thalassemia major:
Marrow expansion ->
"Crew cut" appearance on X-ray
What is the structure of HbF?
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.
Three causes of megaloblastic anemia:
Common finding on blood smear in megaloblastic anemia (other than big-ass RBC):
Anemia in which methylmalonic acid is normal:
Anemia in which methylmalonic acid is elevated:
Anemia 2/2 folate deficiency
Anemia 2/2 B12 deficiency
Four common causes of B12 deficiency:
Which of the macrocytic anemias would you expect to see neurologic sx with?
B12 deficiency anemia
This macrocytic anemia is incurable by folate / B12 administration:
Anemia 2/2 orotic aciduria
What is the treatment for orotic aciduria?
In B12 and folate deficiency anemias, what is the homocysteine level like?
What is the protein responsible for binding ferroportin in anemia of chronic disease?