Deck 8: The Normochromic Anemias Caused by 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

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

Answer 2

A

Question 3

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

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

Answer 3

A

Question 4

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

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

Question 5

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 6

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

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

Question 7

In sickle cell anemia, _____is substituted for glutamine.

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

Question 8

A 26-year-old day laborer with a long history of anemia has a several-month 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  10⁹/L
RBC 2.9  10¹²/L
Hgb 9.5 g/dL
Hct 30%
MCV 103 fL
MCH 31 pg
MCHC 32%
NRBC 15/100 WBC
Platelets 275  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 9

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 10

The Benin haplotype of sickle cell disease is prevalent in which country or countries?

A) Saudi Arabia and Asia
B) Senegal
C) West Africa
D) Central and South Africa

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

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 13

Autosplenectomy is characteristic of:

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

Question 14

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

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

Question 15

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 cell membrane.The most probable diagnosis is:

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

Question 16

Alkaline electrophoresis will not separate hemoglobin C from:

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

Question 17

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

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

Question 18

The hemolytic anemias are generally classified morphologically as:

A) Macrocytic, normochromic
B) Normochromic, normocytic
C) Microcytic, hypochromic
D) Microcytic, normochromic

Question 19

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 20

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 21

is the second most common hemoglobin variant worldwide.

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

Question 22

Which of the following is an alpha chain variant?

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

Question 23

The cell at the tip of the pointer would be described as a/an:

A) Acanthocyte
B) Elliptocyte
C) Sickle cell
D) Stomatocyte