Sickle cell anemia


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  • Sickle cell anemia

    1. 1. • Autosomal recessive disorder characterized byreplacement of the amino acid valine in one ofthe B chains by glutamic acid.
    2. 2. Pathophysiology• Hb S  deoxygenated  polymerization ofHb S molecules  rigid strand of hemoglobinmolecule  sickling of RBCs.
    3. 3. Sickled RBCsAnemia Vascular occlusions
    4. 4. Clinical manifestations of sickle cell anemiaSickle cell anemiaChronichemolytic anemiaSickle cell crises
    5. 5. Chronic hemolytic anemia• Anemia.• Jaundice.• Gall stones.• Hepato-splenomegaly (due to extramedullaryhematopoiesis).
    6. 6. Sickle cell crisesVaso-occlusivecrisesSplenic sequestrationcrisisAplasticcrisisHemolyticcrisis
    7. 7. Vaso-occlusive crises
    8. 8. Stroke
    9. 9. Acute chest syndromeIt is a clinical syndrome characterized by• Chest pain.• Cough• Dyspnea.• Fever.Due to:Chest infection.Fat embolism (secondary to bone infarcts).Pulmonary thrombosis.
    10. 10. Acute abdominal pain• Due to splenic infarct.
    11. 11. Priapism• Painful sustained unwanted erection.• Due to veno-occlusion resulting in bloodtrapping in erectile Priapism tissue.Priapus
    12. 12. Dactylitis• Painful swollen fingers or toes due to boneinfarction.
    13. 13. Splenic sequestration crisis• Acute painful enlargement of the spleen.• Associated with drop in RBCs & plateletcounts.• Due to sudden pooling of large amounts ofblood into the spleen.
    14. 14. Aplastic crisis• Acute reticulocytopenia triggered bypapovirus B19.
    15. 15. Hemolytic crisis• Acute acclerated drop in hemoglobin level.• Occur in patients with co-existent G6PDdeficiency.
    16. 16. Chest• Lung:• Consolidation due to chestinfection.• Heart:• Cardiomegaly and heart failure dueto increased cardiac out put.• Mediastinum:• Posterior mediastinal mass lesiondue to extramedullary hemopoiesis.
    17. 17. Spleen• Splenic infarct.• Autosplenectomy (small calcified spleen).
    18. 18. LiverHepatomegaly:• Due to extramedullary hemopiesis.Hepatic siderosis:• due to repeated transfusion & iron overload
    19. 19. Gall bladder• Gall stones.• Biliary sludge.
    20. 20. Kidney• Renal papillary necrosis.• Renal infarcts.
    21. 21. Skeletal manifestations of sickle cellanemia• Bone marrow hyperplasia.• Bone infarction.• Bone infection.• Growth disturbances.
    22. 22. Bone marrow hyperplasia• Less sever than thalassemia.
    23. 23. Long bones• Diffuse osteoporosis,• Medullary expansion,• Cortical thinning,• Coarse trabeculations (honey comb)• (reactive sclerosis of the secondary trabeculae afterdestruction of the primary trabeculae),• Endosteal apposition, (another response todestruction of the medullary trabeculae by inwardcortical thickening, then cortical splitting giving bonewithin bone appearance). &• Wide vascular channels.
    24. 24. • MRI:• Diffuse low signal on T1 & T2 WIs• (due to persistent red marrow).• Bone scan:• Diffuse symmetrical increased uptake.
    25. 25. Skull
    26. 26. Skull
    27. 27. Bone infarction
    28. 28. Hand & foot syndrome
    29. 29. Bone infection• Salmonella osteomyelitis.• Salmonella spondylodiscitis.
    30. 30. Premature fusion of the epiphysis
    31. 31. Premature fusion of the epiphysis &osteomyelitis