Thalassemia
Background
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↓ synthesis of alpha or beta chains of hemoglobin → precipitation of unpaired chains & premature RBC destruction
Considerations
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Chronic hemolytic anemia:
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Compensation: ↑ cardiac output, ↑ 2,3-DPG, ↑ plasma volume
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Cholilithiasis, splenomegaly
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Sequelae of multiple transfusions/iron overload:
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Cardiomyopathy, dysrhythmias
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Pulmonary hypertension
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Hepatic fibrosis, diabetes
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Alloimmunization
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4 types:
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Beta thalassemia major:
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Potential difficult airway due to maxillary overgrowth (from bone marrow stimulation)
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Hemochromatosis (deposition of hemosiderin into cardiac muscle → dilated cardiomyopathy, heart failure, conduction delays)
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Jaundice secondary to hemolysis
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Hemolytic anemia
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Thinning of cortical bone → potentially difficult regional secondary to vertebral destruction
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Beta thalassemia minor:
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Mild hemolytic anemia & iron deficiency
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Alpha thalassemia major: incompatible with life
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Alpha thalassemia minor: mild anemia
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Goals
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Ensure not anemic for surgery (typical goal hemoglobin > 100)
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Anticipate difficult airway in beta-thalassemia major