Deck 8: The Normochromic Anemias Due to Hemoglobinopathies

Question 1

In hemoglobin C disease, ____ is substituted for glutamic acid.

A)valine
B)adenine
C)lysine
D)leucine

Answer 1

C

Question 2

Painful swelling of hands and feet caused by sickle cells in the microcirculation of infants is termed:

A)Dactylitis
B)Necrosis
C)Apoptosis
D)Fibrosis

Answer 2

A

Question 3

Alkaline electrophoresis will not separate hemoglobin C from:

A)Hemoglobin A₂
B)Hemoglobin F
C)Hemoglobin S
D)Hemoglobin A

Answer 3

A

Question 4

Mr.Bennett was admitted with a hematocrit of 28%.He has a history of chronic anemia, moderate splenomegaly, and recent retinal hemorrhages.His peripheral smear reveals target cells, and some show structures jutting from the red blood cell membrane.The most probable diagnosis is:

A)Hgb S-S
B)Hgb A-C
C)Hgb S-C
D)Hgb A-A

Question 5

The hemoglobin that cannot be separated from Hgb S through electrophoresis in buffer at pH 8.6 is:

A)Hgb D
B)Hgb C
C)Hgb A
D)Hgb F

Question 6

In hemoglobin C disease, the majority of the red blood cells appear as:

A)Target cells
B)Spherocytes
C)Elliptocytes
D)"Bars of gold"

Question 7

Autosplenectomy is characteristic of:

A)Sickle cell trait
B)Hgb C disease
C)Thalassemia
D)Sickle cell anemia

Question 8

Which of the following "poikilocytes" are classically associated with hemoglobinopathies and liver disease?

A)Spherocytes
B)Sickle cells
C)Acanthocytes
D)Target cells

Question 9

Patients with sickle cell anemia generally have a hemoglobin value between:

A)2 and 4 g/dL
B)6 and 8 g/dL
C)8 and 10 g/dL
D)10 and 12 g/dL

Question 10

The lungs are particularly susceptible in individuals with sickle cell disease and may be susceptible to:

A)COPD
B)Pulmonary hypertension
C)Aplastic crisis
D)Cardiac crisis

Question 11

Sickle cell disease is best differentiated from sickle cell trait by:

A)Wright smear examination
B)Determination of fetal hemoglobin
C)Genetic testing
D)Hemoglobin electrophoresis

Question 12

In patients with sickle cell trait and no complicating disease:

A)NRBCs and polychromasia are usually seen on the peripheral smear
B)Sickle cells are prevalent in the peripheral blood
C)There may be occasional hematuria
D)There is a severe decrease in Hgb, Hct, and RBC count

Question 13

A 26-year-old day laborer with a long history of anemia has a several months' history of dyspnea, fever, and pulmonary pain.Additionally, he complains of tightness in the chest and upper abdominal region.His hematology values are as follows: WBC 15.9 $\times$ 10⁹/L
RBC 2.9 $\times$ 10¹²/L
Hgb 9.5 g/dL
Hct 30%
MCV 103 fL
MCH 31 pg
MCHC 32%
NRBC 15/100 WBC
Platelets 275 $\times$ 10⁹/L
Reticulocytes 16.8%
The hemoglobin electrophoresis results are as follows:
Hgb A =3%
Hgb A₂ = 2%
Hgb S = 85%
Hgb F = 10%
The most likely diagnosis is:

A)Sickle cell trait
B)Sickle cell disease
C)Beta thalassemia minor
D)Sickle thalassemia

Question 14

In sickle cell anemia, _____is substituted for glutamine.

A)valine
B)lysine
C)adenine
D)leucine

Question 15

____ is the second most common hemoglobin variant worldwide.

A)Hemoglobin S
B)Hemoglobin E
C)Hemoglobin C
D)Hemoglobin D

Question 16

A patient with sickle cell anemia may develop cardiopulmonary disease due to:

A)Untreated leg ulcers
B)High blood viscosity
C)Increased Hgb F levels
D)Hemolytic crisis

Question 17

A blood smear from a patient with sickle cell crisis shows 80 nucleated red blood cells per 100 leukocytes.The total leukocyte count is 18 $\times$ 10⁹/L.The corrected white blood cell count is:

A)17,200
B)9,000
C)10,000
D)13,400

Question 18

When the bone marrow temporarily ceases to produce cells in a sickle cell patient, a(n) _________ crisis has occurred.

A)aplastic
B)hemolytic
C)vaso-occlusive
D)cardiac

Question 19

A male with sickle cell trait and a female sickle cell trait have what likelihood of having a child with sickle cell anemia?

A)10%
B)25%
C)50%
D)100%

Question 20

The hemoglobin variant that is seen frequently in the Southeast Asian population and migrates with Hgb C at pH 8.6 is:

A)Bart's
B)F
C)E
D)H

Question 21

Which of the following hemoglobins contain an alpha chain variant?

A)Hemoglobin C
B)Hemoglobin G
C)Hemoglobin S
D)Hemoglobin A₂

Question 22

The principle behind hemoglobin electrophoresis is best represented by which of the following statements?

A)Normal hemoglobin does not precipitate in high-molarity buffered phosphate solutions.
B)Hemoglobins migrate to different locations depending on time, pH, and media used.
C)In a pH range of 3 to 10, hemoglobins achieve their isoelectric point.
D)Hematology analyzers count nucleated red blood cells as white blood cells.

Question 23

The clinical picture of a patient with Hgb S-beta-thalassemia is similar to that seen in which other hemoglobinopathy?

A)Sickle cell anemia
B)Sickle cell trait
C)Hgb C disease
D)Hgb E disease