Hemoglobinopathies and Thalassemias

In sickle cell disease the abnormality is related to

the rate of synthesis of hemoglobin

an abnormal molecular structure of hemoglobin

an acquired defect

a membrane dysfunction

Correct! Wrong!

Deoxyhemoglobin C has

decreased solubility

increased solubility

the ability to form intracellular crystals

both A and C

Correct! Wrong!

The incidence of Hb E hemoglobinopathy is highest in

Southeast Asia

China

Vietnam

Native Americans

Correct! Wrong!

What estimated percentage of black Americans are heterozygous for Hb S?

12%

More than 25%

Correct! Wrong!

If a patient with sickle cell anemia is in an acute crisis state, peripheral blood smears may exhibit

leptocytes

drepanocytes

ovalocytes

stomatocytes

Correct! Wrong!

Most unstable hemoglobins

are inherited autosomal dominant disorders

result from amino acid substitutions or deletions

are hemoglobin variants

all of the above

Correct! Wrong!

What factors contribute to the sickling of erythrocytes in sickle cell anemia crisis?

Increase in blood pH and increase in oxygen

Extremely hot weather

Extremely reduced oxygen and increased acidity in the blood

Sickling is spontaneous

Correct! Wrong!

The peripheral blood smear in silent state patients with a-thalassemia typically appears as

normochromic, normocytic

microcytic, hypochromic

macrocytic, normocytic

macrocytic, hypochromic

Correct! Wrong!

In a-type thalassemia, with three inactive a genes, which of the following is characteristic?

Hb A2

Hb A

Hb H

Hb F and A2

Correct! Wrong!

What is the primary risk to thalassemia major patients who receive frequent and multiple blood transfusions?

Iron overload

Citrate toxicity

Polycythemia

Hyperviscosity

Correct! Wrong!

One of the two most common monogenetic diseases of man is

sickle cell trait

sickle cell anemia

a-thalassemia

Hb SC disease

Correct! Wrong!

Homozygous b-thalassemia patients have

no manifestations of anemia

only mild anemia

moderate anemia

severe transfusion-dependent anemia

Correct! Wrong!

In the hemoglobinopathies, a trait is described as

heterozygous and asymptomatic

heterozygous and symptomatic

homozygous and asymptomatic

homozygous and symptomatic

Correct! Wrong!

In sickle cell anemia the cause is

a change of a single nucleotide (GAT to GTT)

the substitution of valine for glutamic acid at the sixth position on the beta chain of the hemoglobin molecule

not genetic

both A and B

Correct! Wrong!

The characteristic hemoglobin concentration in a patient’s silent state with heterozygous b-thalassemia is

Hb A level normal

Hb A2 increased

Hb A2 level decreased

Hb F level increased

Correct! Wrong!

Hemoglobinopathies can be classified as

abnormal hemoglobin globulin structure

a defect of hemoglobin globulin synthesis

a combination of defects of both structure and synthesis

all of the above

Correct! Wrong!

Thalassemias are characterized by

abnormal amino acid sequence of the hemoglobin molecules

defective iron synthesis

absence or decrease in synthesis of one or more globlin subunits

skeletal membrane defects

Correct! Wrong!

Normal adult hemoglobin contains the following components: Hb A (95% to 98%), Hb A2 (2% to 3%), Hb A1 (3% to 6%), and Hb F (<1%).

True

Fals

Correct! Wrong!

The most common complaint associated with sickle cell anemia?

Acute pain

Organ or tissue failure

Stroke

All of the above

Correct! Wrong!

The common denominator in the hemoglobinopathies is that all are

structural defects in the erythrocyte membrane

metabolic defects in the erythrocytic physiology

inherited or genetic defects related to hemoglobin

acquired defects related to hemoglobin

Correct! Wrong!

Hemoglobinopathies and Thalassemias

Fair

Good

Very good

Hemoglobinopathies and Thalassemias

In sickle cell disease the abnormality is related to

Deoxyhemoglobin C has

The incidence of Hb E hemoglobinopathy is highest in

What estimated percentage of black Americans are heterozygous for Hb S?

If a patient with sickle cell anemia is in an acute crisis state, peripheral blood smears may exhibit

Most unstable hemoglobins

What factors contribute to the sickling of erythrocytes in sickle cell anemia crisis?

The peripheral blood smear in silent state patients with a-thalassemia typically appears as

In a-type thalassemia, with three inactive a genes, which of the following is characteristic?

What is the primary risk to thalassemia major patients who receive frequent and multiple blood transfusions?

One of the two most common monogenetic diseases of man is

Homozygous b-thalassemia patients have

In the hemoglobinopathies, a trait is described as

In sickle cell anemia the cause is

The characteristic hemoglobin concentration in a patient’s silent state with heterozygous b-thalassemia is

Hemoglobinopathies can be classified as

Thalassemias are characterized by

Normal adult hemoglobin contains the following components: Hb A (95% to 98%), Hb A2 (2% to 3%), Hb A1 (3% to 6%), and Hb F (<1%).

The most common complaint associated with sickle cell anemia?

The common denominator in the hemoglobinopathies is that all are

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