Hemolytic Anemia

1. Hereditary

2. Aquired

3. Enzyme Defenciency: G6PD deficiency, Pyruvate kinase.

4. Hemoglobinopathies: Sickle Cell Anemia & thalassemias

5. Membrane Abnormalities: hereditary spherocytosis

6. Immune-mediated: Coomb' Test (positive)

7. Autoimmune hemolytic anemia (AIHA)

8. Cold AIHA: IgM Antibodies bind to RBC's at 4 degrees Celsius Etiologies: Idiopathic. Infection: mycoplasma pneumonia, infectious mononucleosis, Cytomegalo Virus, Human immunodeficiency virus. Drugs- methyldopa and penicillin. Paraoxysmal Nocturnal Hemoglobinuria

9. Warm AIHA: IgG Antibodies opsonize RBC's at 37 degrees Celsius Etiologies: idiopathic, lymphoproliferate (Chronic Lymphocytic Leukemia, Non-Hodgkin Lymphoma), autoimmune diseases (Systemic Lupus Erythematosus)

10. Risk Factor: glucose-6-phosphate dehydrogenase (G6PD) deficiency mostly affects males of African or Mediterranean descent. In the United States, the condition is more common among African Americans than Caucasians.

11. Non immune-mediated: Coombs' Test (negative)

12. Traumatic: Microangiopathic Hemolytic Anemia (MAHA) includes: Thrombotic Thrombocytopenic Purpura, Hemolytic Uremic Syndrome, Disseminated Intravascular Coagulation. Protheses: valves, transjugular intrahepatic portosystemic shunt (TIPS)

13. Direct Infections: Malaria, Clostridia, animal/insect bite, etc. Toxins: Wilson's Disease

14. Entrapment: Hypersplenism

15. Drugs: ribavarin

16. Diagnostic Tests: CBC- low Hg and Hct, High reticulocyte count; Chem- low haptoglobin, high LDH; Urinalysis- hemoglobinuria, hemosiderinuria; Peripheral blood smear: Heinz bodies and bite cells with G6PD deficiency. Sickle-shaped RBC's with Sickle-cell anemia. Spherocytes with Hereditary spherocytosis. Schistocytes with MAHA.

17. Symptoms include: paleness of the skin fatigue fever confusion lightheadedness dizziness weakness or inability to do physical activity Advanced signs and symptoms: dark urine jaundice heart murmur increased heart rate enlarged spleen enlarged liver

18. Treatments: Blood Transfusions; Medications (corticosteroids, immunosuppressants- rituximab, cyclosporine, hydroxyurea for sickle cell pts); Plasmapheresis; Surgery; Blood and Marrow Stem Cell Transplant; Lifestyle Changes (Low temperature avoidance, AIHA with cold-reactive antibodies)