Deck 7: Normochromic Anemias: Biochemical and Membrane Disorders and Miscellaneous Red Cell Disorders

Question 1

Which of the following is most closely associated with G6PD deficiency?

A)Continuous intravascular hemolysis occurs.
B)G6PD deficiency is a sex-linked inherited disease.
C)Splenectomy is commonly done to control symptoms.
D)The majority of the erythrocytes in these individuals are microcytic.

Answer 1

B

Question 2

The inability of the red blood cell to generate ATP for membrane function is most closely associated with:

A)Pyruvate kinase deficiency
B)G6PD deficiency
C)Fanconi's anemia
D)Congenital hemolytic anemia

Answer 2

A

Question 3

The deficit of which of these is most closely associated with Southeast Asian ovalocytosis?

A)Spectrin
B)Ankyrin
C)Molecular band 3
D)G6PD

Answer 3

C

Question 4

Which of the following red blood cell membrane defects results in an increased sensitivity to complement lysis?

A)Cold agglutinin syndrome
B)Paroxysmal nocturnal hemoglobinemia
C)Diamond Blackfan anemia
D)Hereditary spherocytosis

Question 5

In hereditary spherocytosis, the reticulocyte count will typically be:

A)Less than 0.5%
B)0.5% to 1.5%
C)3% to 10%
D)Greater than 10%

Question 6

An anemia that manifests as decreased marrow cellularity, pancytopenia, and a reticulocytopenia is appropriately termed:

A)Megaloblastic anemia
B)Aplastic anemia
C)Sideroblastic anemia
D)Iron deficiency anemia

Question 7

One of the least severe clinical manifestations of G6PD deficiency is:

A)Acute hemolytic anemia
B)Favism
C)Neonatal jaundice
D)Congenital non-spherocytic hemolytic anemia

Question 8

Spherocytes may enter the circulation via a variety of mechanisms.To distinguish the CBC of a patient with hereditary spherocytosis from the CBC of a patient with autoimmune hemolytic anemia, one could use the:

A)MCHC
B)Hematocrit
C)MCH
D)MCV

Question 9

Bite cells are formed from:

A)The spleen "pitting" out Heinz bodies
B)Third-degree burns
C)Spectrin deficiency
D)G6PD deficiency

Question 10

Spherocytes do not function well in the splenic environment because the:

A)Osmotic environment of the spherocyte is impaired
B)Immunologic function of the spleen is impaired
C)Reservoir function is impaired
D)Filtration function of the spleen is compromised

Question 11

Individuals with Fanconi's anemia characteristically will have:

A)Intravascular hemolysis
B)Increased Hgb F
C)Ringed sideroblastics
D)Thrombocytosis

Question 12

Red blood cells that are rigid, provide resistance to malaria, and are spoon shaped are termed:

A)Burr cells from kidney disease
B)Spherocytes from hereditary spherocytes
C)Ovalocytes from Southeast Asian ovalocytosis
D)Sickle cells from sickle cell disease

Question 13

A defect in stomatin is the main cause of hereditary spherocytosis.

Question 14

Spherocytes differ from normal cells in all of the following ways except having:

A)Decreased surface-to-volume ratio
B)Decreased central pallor
C)Decreased resistance to hypotonic saline
D)Increased deformability

Question 15

The RBC inclusions that are composed of denatured hemoglobin are called:

A)Pappenheimer bodies
B)Howell-Jolly bodies
C)Heinz bodies
D)Siderotic granules

Question 16

An eccentric "puddling" of a red blood cell's hemoglobin contents is most closely associated with:

A)Stomatocytosis
B)Xerocytosis
C)G6PD deficiency
D)Pyruvate kinase deficiency

Question 17

Cholelithiasis is a frequent complication of patients with hereditary spherocytosis because:

A)Spherocytes are an abnormal red blood cell morphology
B)The gallbladder does not function well in this condition
C)Continued hemolysis in this condition causes gallstones to form
D)The filtration function of the spleen is overworked

Question 18

An osmotic fragility test is performed on a patient's sample.Initial hemolysis occurs at 0.75% NaCl and complete hemolysis occurs at 0.50% NaCl.These results are indicative of which of the following disorders?

A)Sickle cell anemia
B)Hgb C disease
C)Thalassemia major
D)Hereditary spherocytosis