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34 Cards in this Set
- Front
- Back
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The common denominator in the hemoglobinopathies is that all are:
A.) structural defects in the erythrocyte membrane. B.) metabolic defects in the erythrocytic physiology C.) inherited or genetic defects related to hemoglobin D.) acquired defects related to hemoglobin |
C.) inherited or genetic defects related to hemoglobin
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Hemoglobinopathies can be classified as:
A.) abnormal hemoglobin globulin structure B.) a defect of hemoglobin globulin synthesis C.) a combination of defects of both structure and synthesis D.) all of the above |
D.) all of the above
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Normal adult hemoglobin contains the following components: HbA (95%-98%), HbA2 (2-3%), HbA1 (3-6%) and HbF (<1%).
A.) True B.) False |
A. True
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In the hemoglobinopathies, a trait is described as:
A.) heterozygous and asymptomatic B.) heterozygous and symptomatic C.) homozygous and asymptomatic D.) homozygous and symptomatic |
A.) heterozygous and asymptomatic
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In sickle cell anemia, the cause is:
A.) a change of a single nucleotide (GAT to GTT). B.) the substitution of valine for glutamic acid at the sixth position on the beta chain of the hemoglobin molecule C.) not genetic D.) both A and B. |
D.) both A and B
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In sickle cell disease the abnormality is related to:
A.) the rate of synthesis of hemoglobin B.) an abnormal molecular structure of hemoglobin C.) an acquired defect D.) a membrane dysfunction |
B.) an abnormal molecular structure of hemoglobin
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The most common inherited hematatological disease is:
A.) sickle cell trait B.) sickle cell anemia C.) sickle-beta thalassemic D.) hemoglobin SC disease |
B.) sickle cell anemia
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If a patient with sickle cell anemia is in an acute crisis state, peripheral blood smears may exhibit:
A.) leptocytes B.) drepanocytes C.) ovalocytes D.) stomatocytes |
B.) drepanocytes
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What estimated percentage of black Americans are heterozygous for hemoglobin S?
A.) 4% B.) 8% C.) 12% D.) More than 25% |
B.) 8%
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What factors contribute to the sickling of erythrocytes in sickle cell anemia crisis?
A.) increased in blood pH and increase in oxygen B.) decrease in blood pH and decrease in oxygen C.) extremely reduced oxygen and increased aciditiy in the blood D.) sickling is spontaneous |
C.) extremely reduced oxygen and increased acidity in the blood
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What clinical signs and symptoms are associated with sickle cell anemia?
A.) painful crisis B.) organ or tissue failure C.) stroke D.) all of the above |
D.) all of the above
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Thalassemias are characterized by:
A.) abnormal hemoglobin molecules B.) defective alpha globulin structure C.) abnormality in the rate of synthesis D.) skeletal membrane defects |
C.) abnormality in the rate of synthesis
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Homozgous beta-thalassemia patients have:
A.) no manifestations of anemia B.) only mild anemia C.) moderate anemia D.) severe transfusion-dependent anemia |
D.) severe transfusion-dependent anemia
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In alpha type thalassemia, with three inactive alpha genes, which of the following is characteristic?
A.) Hb A2 B.) Hb A C.) Hb H D.) Hb F and A2 |
C.) Hb H
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What is the primary risk to thalassemia major patients who receive frequent and multiple blood transfusions?
A.) Iron overload B.) Citrate toxicity C.) Polycythemia D.) Hyperviscosity |
A.) iron overload
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The peripheral blood smear in silent state patients with alpha thalassemia typically appears as:
A.) normochromic, normocytic B.) microcytic, hypochromic C.) macrocytic, normocytic D.) macrocytic, hypochromic |
A.) normochromic, normocytic
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The characteristic hemoglobin concentration in a patient's silent state with heterozygous beta-thalassemica is:
A.) hemoglobin A level-normal B.) hemoglobin A2 increased C.) hemoglobin A2 level decreased D.) hemoglobin F level increased |
D.) hemoglobin F level increased
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Deoxyhemoglobin C has:
A.) decreased solubility B.) increased solubility C.) the ability to form intracellular crystals D.) both A and C |
D.) both A and C
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The incidence of hemoglobin E hemoglobinopathy is highest in:
A.) southeast Asia B.) China C.) Vietnam D.) Native Americans |
A.) southeast Asia
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Most unstable hemoglobins:
A.) are autosomal dominant disorders B.) result from amino acid substitutions or deletions C.) are hemoglobin variants D.) all of the above |
D.) all of the above
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Three major categories of classification of hemoglobin defects
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1.) abnormal molecular structure
2.) defect in rate of synthesis 3.) combination of abnormal molecular structure with a synthesis defect |
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Etiology of sickle cell disease
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- inherited from both parents
- leading to polymerization when deoxygenated |
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Sickle cell MCHC
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increased
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Vaso-occlusion
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- adherence of sickled cells to vascular endothelium
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Sickle cell anemia blood smear
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- hypochromia, target, microcytes, polychromatophilia, and basophilic stippling
- if acute crisis, then drepanocytes (sickle cells) |
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Sickle cell tests
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- hemoglobin electrophoresis
- alkali denaturation test - acid elution test - osmotic fragility test |
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sickle cell anemia reticulocytes
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reticulocytosis
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sickle cell MCV
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increased
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sickle cell unconjugated bilirubin and methemalbumin
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increased
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sickle cell haptoglobin and hemopexin
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decreased
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sickle cell lactic dehydrogenase (LDH)
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increased
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sickle-beta thalassemia
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- the inheritance of the sickle gene from one parent and a beta-thal gene from another
- mod severe hemolytic anemia - splenomegaly - can make small amount of Hb A |
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sickle-C disease
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- no HbA
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sickle cell trait
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- contain HbS and A
- more survival |
