Thalassemia

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Thalassemia

  1. 1. Dr / Hytham NafadyThalassemia
  2. 2. it is a genetic disorder ofhemoglobin synthesis in whichthere is defective synthesis ofalpha or beta chains.
  3. 3. ThalassemiaAlpha Thalassemia Beta thalassemiaDefective synthesis ofalpha chainDefective synthesis ofBeta chain
  4. 4. ThalassemiaThalassemia major Thalassemia minorHomozygossever formHeterozygosminor form
  5. 5. Pathogenesis of thalassemiaineffective erythropoiesis
  6. 6. C.PHemolytic anemia:Anemia.Jaundice.Hepatosplenomegally due to extramedullaryhemopoiesis.Rodent facies (prominent malar eminences &malalignement of the teeth) due to facial bonedeformity 2ry to bone marrow hyperplasia.
  7. 7. Bone marrow hyperplasiaLong bones:Diffuse osteoporosis,Medullary expansion,Cortical thinning,Coarse trabeculations (reactive sclerosis of the 2ry bonetrabeculae after destruction of the primary trabeculae), &Enlargement of the nutrient foramina.
  8. 8. Bone marrow hyperplasiaSkull:Widening of the diploic space.Thinning of the inner & outer table (complete resorption of the outertable may occur).Prominent 2ry trabeculae (hair on end appearance).The occipital bone is spared as it lacks hemopoietic bone marrow.
  9. 9. Hair on end appearance
  10. 10. Facial bones:Hypopneumatization of the frontal, maxillary & sphenoidsinuses (which are filled with marrow containing bone).The ethmoid sinuses are spared.Prominent malar eminences.Anterior & medial displacement of the developing teeth.
  11. 11. Spine:Diffuse osteoporosis.Cortical thinning.Coarse trabecular pattern.Platyspondyly.
  12. 12. Ribs:Diffuse osteoporosis.Medullary expansion.Cortical erosions.Bullous enlargement of the posterior ribs.
  13. 13. Red marrow reconversion
  14. 14. Extramedullary hematopoiesis
  15. 15. 2ry hemochromatosisReticulo-endothelial iron deposition
  16. 16. Skeletal dysplasia due to DFX TTMetaphyseal bands & growth arrest lines
  17. 17. Skeletal dysplasia dueto DFX TT
  18. 18. DD of growth arrest linesAnemia (thalassemia & sickle cell anemia).Lead poisoning.Chemotherapy.Treated leukemia.Healing rickets.Healing hyperparathyroidism.

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