Hematology in Practice - Chapter 8
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| The hemoglobin variant that is seen frequently in the Southeast Asian population, and migrates with Hgb C at pH 8.6, is: | E
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| A patient with sickle cell anemia may develop cardiopulmonary disease due to: | High blood viscosity
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| The Benin haplotype of sickle cell disease is prevalent in which country or countries? | West Africa
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| What "poikilocytes" are classically associated who hemoglobinopathies and liver disease? | Target cells
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| Painful swelling of hands and feet caused by sickle cells in the microcirculation of sickle cells in infants is termed: | Dactylitis
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| Patients with sickle cell anemia generally have a hemoglobin value between: | 6 and 8 g/dL
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| Alkaline electrophoresis will not separate hemoglobin C from: | Hemoglobin A2
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| _____ is the second most common hemoglobin variant worldwide. | Hemoglobin E
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| The qualitative hemoglobinopathies cause disease by producing hemoglobin chains that are: | structurally altered
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| What clinical feature of sickle cell disease accounts for the highest number of hospital admissions? | Vaso-occlusion
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| What percentage of African Americans have heterozygous sickle cell trait? | 8%
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| What is the probable etiologic association between malaria and sickle cell trait? | Sickle genes offers some protection against malaria.
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| A patient has target cells and a positive tube solubility test. This patient probably: | has either sickle cell anemia or trait
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| Moderate to marked target cells are found on a blood smear. Which of the following can most likely by eliminated? A. hemoglobin C disease B. Hereditary spherocytosis C. Hemoglobin E disease D. liver disease | B. Hereditary spherocytosis
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| What is a typical finding in patients with hemoglobin M? | Elevated methemoglobin
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| What is the correct relationship between red cells' affinity for oxygen and red cell production? | When affinity is high, less oxygen is released and the body compensates by increasing erythropoietin and making more red cells.
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| What is the amino acid substitution in patients with sickle cell anemia? | Valine for glutamic acid
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| What factor contributes to the pathophysiology of sickling? | Dehydration
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| What statement pertains to most clinically significant hemoglobin variants? | Most are single amino acid substitutions
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| What hemoglobin ranks second in variant hemoglobins worldwide? | Hgb E
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| Which shows crystals appearing like bars of gold in the peripheral smear? | Hgb CC disease
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| What condition is the leading cause of hospitalization for sickle cell patients? | Acute chest syndrome
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| What hemoglobin separation method is used for most newborn hemoglobin screening? | Isoelectric focusing
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| Autosplenectomy is characteristic of: | Sickle cell anemia
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| The Benin haplotype of sickle cell disease is prevalent in which country or countries? | West Africa
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| When the bone marrow temporarily ceases to produce cells in a sickle cell patient, an ______ crisis has occurred. | Aplastic
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