DR Medhat MRCP

• Management :
1) Phlebotomy (venesection) : removing 400-500 ml of blood (200-250 mg iron) weekly
or every two Weeks + good hydration before & after
procedure.
2) Fe –chelating agents : deferroxamine (desferal).
3) Monitoring adequacy of venesection:
سؤال choice) 1) Transferrin saturation should be kept below 50% (test of
2) Serum ferritin concentration should be kept below 50 ug/l'.
III. Sideroblastic anaemia
- Is a condition where red cells fail to completely form haem, whose biosynthesis takes
place partly in the mitochondria.
- This leads to deposits of iron in the mitochondria that form a ring around the nucleus
called a ring sideroblast.
- It may be congenital or acquired
• Causes :
Congenital cause :
delta-aminolevulinate synthase-2 deficiency.
Acquired causes :
1. Myelodysplasia.
2. Alcohol
3. Lead
4. Chloramphenicol & Anti-TB medications
(rifampicin & INH).
• Investigations :
Hypochromic microcytic anaemia (more so in congenital)
Bone marrow smear :
• Management
Supportive
Sideroblasts and increased iron stores.
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