Normochromic Anemias

Question 1

What is the cause of Hereditary Spherocytosis?

Answer 1

Deficiency in Spectrin & Ankyrin
Protein band 3
Protein band 4.2

Question 2

What test do we use to diagnose Hereditary Spherocytosis?

Answer 2

Osmotic Fragility Test
Normal RBC’s hemolyze at 0.45% NaCl
Spherocytes hemolyze at 0.65% NaCl

Question 3

A deficiency in the protein Stomatin is a cause of what?

Answer 3

Hereditary Stomacytosis
Smiling Cell

Question 4

A deficiency in the protein Spectrin is a cause of what?

Answer 4

Hereditary Elliptocytosis

Question 5

Where do we tend to see more severe Hereditary Elliptocytosis?

Answer 5

In infants
As they age, turns into mild HE

Question 6

What does a deficiency in Band 3 cause?

Answer 6

Southeast Asian Ovalocytosis
Provides some protection against malaria

Question 7

What do we see in a peripheral smear of Spherocytic Hereditary Elliptocytosis?

Answer 7

Spherocytes and elliptocytes

Question 8

What do we see when a patient is diagnosed with Hereditary Pyropoikilocytosis?

Answer 8

Red cell budding
Cell fragmentation at lower temperatures

Question 9

What is the cause of Pyruvate Kinase Deficiency?

Answer 9

RBC’s unable to generate ATP due to the lack of enzyme

Question 10

Why is the enzyme G6PD needed in the body?

Answer 10

Prevents oxidative stress
NADPH required to convert glutathione into the reduced state

Question 11

What results within hours of contact of fava beans?

Answer 11

Favism

Question 12

A patient needs to be tested for their G6PD levels. When should we perform a test?

Answer 12

A test should NOT be performed during a hemolytic episode