Anemia II- Krafts

Question 1

What is a hemoglobinopathy?

Answer 1

Qualitative hemoglobin abnormality (can lead to hemolysis and vaso-occlusion)

Question 2

What is the best lab test for hemoglobinopathies?

Answer 2

Hemoglobin electrophoresis

Question 3

What causes sickle cell anemia?

Answer 3

A point mutation in the B chain of hemoglobin that substitutes a valine for glutamate

Question 4

What are some of the clinical findings in sickle cell anemia?

Answer 4

Chronic hemolyisis (due to fragility of sickle RBCs)

Micro-vascular occlusion (can’t bend and stack up on each other)

Leads to ischemia ( like foot ulcers)

Question 5

What would you expect to see in a blood smear of someone with sickle cell anemia?

Answer 5

Sickle cells
Nucleated RBC
Howell-jolly bodies
Pappenheimer bodies
Increased platelet count

Question 6

What are howell jolly bodies and when would you see them?

Answer 6

little remnant of nucleus that don’t get excluded (little black dot in cell)

Seen in sickle cell anemia

Question 7

Chronic hemolysis
micro-vascular occlusion
howell-jolly bodies
nucleated RBCs
increased platelets
pappenheimer bodies

Answer 7

Sickle cell anemia

Question 8

What are the two types of thalasesmia? What is the genetic difference between them?

Answer 8

Alpha thal= DELETION of alpha chains

Beta thal= DEFECTIVE beta chains (problems with transcription, translation, mRNA Processing)

Question 9

what are the two pathologies associated with thalassemia?

Answer 9

Decreased hemoglobin production (because decrease in globin chains)

Excess unpaired alpha chains (in beta-thal) or vis versa - which form tetramers = premature RBC destruction

Question 10

Morphology of thalasemmia?

Answer 10

Microcytic
Hypochromatic
Decreased anisocytosis
Basophilic stippling
Target cells
increased severity = more nucleated RBCs

Question 11

What is glucose 6 phosphate dehydrogenase normally used for?

Answer 11

catalyzes initial step in the pentose phosphatepathway, reduces NADP to NADPH in the process

NADPH is used to keep glutathione in reduced state to deal with oxidative stress

Question 12

In what patients blood slide would you see

Cell fragments
micro-spherocytes
Bite cells?

Answer 12

Glucose 6 Phosphate dehdrogenase deficiency

Question 13

What causes bite cells?

Answer 13

G6PD defiecency
Formation of heinz boides (denatured globin that forms round inclusion body and compromised membrane plasticity)

Heinz bodies are taken out by MACs in the spleen forming bite cells

Question 14

Fragment cells can lead you to what definitive diagnosis?

Answer 14

Microangiopathic Hemolytic Anemia!!!

Question 15

What causes microangiopathic hemolytic anemia?

Answer 15

RBC under go physical trauma, like getting impaled on fibrin strand in microcirulaiton

Question 16

Helmet cells
Triangulocyte
Schistocyte
Keratocyte

Answer 16

Microangiopathic Hemolytic anemia!!!!

Question 17

In what anemia would you see a NORMAL hemoglobin?

Answer 17

Anemia of acute blood loss

Question 18

Normochromatic
Normocytic
"Bland looking"
Decreased serum Fe
Increased Ferritin
DecreasedTIBC

Answer 18

Anemia of chronic disease!!!

Question 19

What causes anemia in chronic renal disease?

Answer 19

Decreased erythropoietin

Question 20

What causes anmeia in chronic liver disease?

Answer 20

Shortened red blood cell survival and impaired bone marrow response

Question 21

In what patients would you echinocytes?

Answer 21

“burrs”

Pts with anemia of chronic renal disease

Question 22

In what patients would you see acanthocytes?

Answer 22

“spurrs” = 5-10 longs, spiky surface projections

Seen in anemia of chronic liver disease

Question 23

What is aplastic anemia?

Answer 23

bone marrow is “empty”
Few hematopoetic precursors
Pancytopenia = all major cell lines (red, white, platelets) are severely decreased