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12 Cards in this Set
- Front
- Back
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What is sickle cell disease?
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Autosomal recessive disorder, an abnormal hemoglobin leads to chronic hemolytic anemia with numerous clinical consequences. Single DNA base change substitution of valine for glutamine.
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What contributes to the formation of sickled red blood cells?
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Hemoglobin S is unstable and polymerizes in the setting of various stressors, including hypoxemia, dehydration and acidosis.
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What are the Symptoms and Signs of sickle cell disease?
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Chronic hemolytic anemia produces jaundice, pigment gallstone, splenomegaly, and poorly healing ulcers.
Life-threatening severe anemia can occur during hemolytic or aplastic crises, generally associated with viral or other infection or by folate deficiency. |
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What organ systems are affected with repeated episodes of vascular occlusion?
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heart, lungs, kidney, bones and liver. Ischemic necrosis of bone occurs, leading to osteomyelitis due to staphylococci or salmonellae.
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What will peripheral blood smear show in sickle cell anemia?
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Sickled red blood cells and target cells. In addition, the red blood cell in the lower central area of the field contains a hard peripheral inclusion called a Howell-Jolly body.
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What is Howell-Jolly body?
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These area nuclear remnants and is the consequence of hyposplenism.
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How do you dx sickle cell anemia?
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Diagnosis of sickle cell anemia is confirmed by hemoglobin electrophoresis.
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What are the treatments when an acute painful episodes occurs?
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Precipitating factors should be identified and infections treated if present. Hydration, and provide oxygen when needed.
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What is the treatment for an Acute vaso-occlusive crises?
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exchange transfusion.
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What is the indication for exchange transfusion?
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Intractable pain crises, priapism, and stroke.
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What is the role of Cytotoxic agents?
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By stimulating erythropoiesis as to increase the hemoglobin F levels. Hydroxyurea (500–750 mg/d) reduce frequency of painful crisis.
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What is special about hemoglobin F?
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hemoglobin F cannot participate in polymer formation, thus its presence markedly retards sickling.
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