Thalassemia

 

 

Background

 

  • ↓ synthesis of alpha or beta chains of hemoglobin  precipitation of unpaired chains & premature RBC destruction 

 

 

Considerations

 

  • Chronic hemolytic anemia:

    • Compensation: ↑ cardiac output, ↑ 2,3-DPG, ↑ plasma volume

    • Cholilithiasis, splenomegaly

  • Sequelae of multiple transfusions/iron overload:

    • Cardiomyopathy, dysrhythmias

    • Pulmonary hypertension

    • Hepatic fibrosis, diabetes

    • Alloimmunization

  • 4 types:

    • ​Beta thalassemia major:

      • ​Potential difficult airway due to maxillary overgrowth (from bone marrow stimulation)

      • Hemochromatosis (deposition of hemosiderin into cardiac muscle → dilated cardiomyopathy, heart failure, conduction delays)

      • Jaundice secondary to hemolysis

      • Hemolytic anemia

      • Thinning of cortical bone → potentially difficult regional secondary to vertebral destruction

    • Beta thalassemia minor:

      • ​Mild hemolytic anemia & iron deficiency

    • Alpha thalassemia major: ​incompatible with life

    • Alpha thalassemia minor​mild anemia

 

 

Goals

 

  • Ensure not anemic for surgery (typical goal hemoglobin > 100)

  • Anticipate difficult airway in beta-thalassemia major

 All rights reserved 2017 © anesthesiaconsiderations.com

 Feedback & inquiries: