Hereditary spherocytosis is the result of a defect in: a. ...

[penguins]

Hereditary spherocytosis is the result of a defect in:
 
  a. Hemoglobin structure
  b. Red cell membrane lipid bilayer
  c. Red cell membrane skeletal proteins
  d. Rate of synthesis of globin chains

Question 2

Which of the following tests can confirm a paroxysmal nocturnal hemoglobinuria diagnosis in a patient?
 
  a. Sucrose hemolysis test (sugar water test)
  b. Reticulocyte count
  c. Ham test (acidified serum lysis test)
  d. Flow cytometry for cell surface markers

[Bison]

Answer to Question 1

ANS: C
Abnormalities in red cell membrane skeletal proteins are present in hereditary spherocytosis.

Answer to Question 2

ANS: D
The reticulocyte count is elevated in all hemolytic anemias at the time of hemolysis (unless an aplastic crisis is present). Of the other procedures listed only flow cytometry is sensitive enough to be useful for the diagnosis of paroxysmal nocturnal hemoglobinuria. The sucrose hemolysis and Ham tests have been replaced by flow cytometry.

[penguins]

Gracias!

[xiaomengxian]

Excellent