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Thalassaemia - Coggle Diagram

- - - - prevented by regular transfusions
      - BM expansion
      - hepatosplenomegaly
      - classical facies
        
        rare to see in developed countries
        
        skull bossing
        
        maxillary overgrowth
  - - - Fe overload
        
        CM + HF
        
        cirrhosis
        
        diabetes
        
        infertility
        
        growth failure + delayed sexual maturation due to fe deposition in pit gland
        
        must give Fe chelator subcut (desferrioxamine) or PO (deferasirox) from 2/3 y/o
        
        skin hyperpigmentation
      - ab formation in 10%
        
        makes finding compatible blood difficult
      - infection
        
        now uncommon
        
        hep A,B,C
        
        HIV
        
        malaria
        
        prions
      - venous access
        
        portacath may be needed
        
        predisposes to infection
    - - only for major with HLA identical sibling
      - 90-95% cure rate
      - but 5% mortality
  - - - look @ ferritin - low in fe deficiency, not in thalassaemia minor