Objectives

After completing this chapter, the student will be able to:

1. Recall the general characteristics of the hemo-globinopathies.

2. Describe the pathophysiology of the sickle disorders.

3. Identify the amino acid substitution in sickle cell disorders.

4. Identify the amino acid substitution in hemoglobin C disease.

5. Describe the inheritance patterns of the sickle disorders.

6. List the clinical and laboratory features of sickle cell anemia, sickle cell trait, hemoglobin C disease, hemoglobin C trait, and hemoglobin SC disease.

7. Review the physiological conditions that most typically affect individuals with sickle cell anemia.

8. List conditions that may precipitate a sickle cell crisis.

9. Recognize normal hemoglobin patterns on hemoglobin electrophoresis at pH 8.6 and 6.2.

10. Recognize abnormal hemoglobin patterns on electrophoresis at pH 8.6 and 6.2.

11. Describe the treatment protocol for patients with sickle cell anemia.

12. Differentiate the clinical and laboratory features of other abnormal hemoglobins such as hemoglobin E C^ D and Gphlla.

13. List the key features of sickle hemoglobin in combination with thalassemias.

14. Calculate the white blood cell correction formula when nucleated red blood cells (nRBCs) are noted in the peripheral smear.

15. Summarize the general principles of acid and alkaline electrophoresis and isoelectric focusing.

114 Part II • Red Cell Disorders

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