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## Sickle Cell Disease ### Clinical Features - Pain - Visual blurring - Jaundice - Syncope - Dactylitis ### Laboratory Findings - Normocytic normochromic anemia with hemoglobin level ranging between 6 and 8 g/dL. - Peripheral blood smear can be striking with: - Sickle cells (common) - Num...

## Sickle Cell Disease ### Clinical Features - Pain - Visual blurring - Jaundice - Syncope - Dactylitis ### Laboratory Findings - Normocytic normochromic anemia with hemoglobin level ranging between 6 and 8 g/dL. - Peripheral blood smear can be striking with: - Sickle cells (common) - Numerous target cells - Fragmented red cells - Polychromasia and nucleated red cells. - Reticulocyte average count 5-20% but will decrease during the aplastic crises. - Leukocytosis and thrombocytosis is common. - Bone marrow: marked erythroid hyperplasia, except during aplastic crises. ### Neck Stiffness And Headache - Neurological Symptoms - Infection - Leg ulcer - Weakness and pallor ### Screening Procedures - **Solubility Test:** Special reagents that precipitate the abnormal Hb S. - **Sodium Metabisulfite:** An old screening test in which Na-metabisulfite induces sickling. It is important to remember that some rare abnormal Hbs also cause sickling, such as variants of HbC. - **Demonstration of Hb S by hemoglobin electrophoresis or HPLC:** This is the definitive test. As the patient with sickle cell anemia produces variant beta chain. The pattern is: Hb S around 80%, Hb F ranges from 1-20%, no Hb A with normal to slightly increased Hb A2. ### Sickle Cell Trait - The peripheral blood smear is usually normal, with the exception of target cells. ### Solubility Screening - Hemoglobin elevated - Sickle cells - Hemoglobin C disease - The disease is characterized by: - Normal glutamic acid is replaced by Lysine in the beta chain. - Hb C migrates more slowly than Hb A in electrophoresis and separates from it. - C tends to form crystals within the red cells.