small lecture about sideroblastic anemia

2. anemia in which the bone marrow produces
ringed sideroblasts rather than healthy red
blood cells (erythrocytes) .
The body has iron available but cannot
incorporate it into hemoglobin, which red
blood cells need to transport oxygen
efficiently

3. atypical, abnormal nucleated erythroblasts
with granules of iron accumulated in
perinuclear mitochondria .
seen in aspirates of bone marrow.
Ring sideroblasts are cells that arrange of the
iron granules in a ring form around the
nucleus

6. Hereditary ??

7. Hereditary ??
AminolevulinicAcid Synthase (ALA)
Deficiency
X – linked Dx

8. Acquired causes :
Primary >>>

9. Acquired causes :
Primary >>>
Idiopathic

10. Acquired causes :
Secondary ?
Toxins >>
Drugs >>
Nutritional >>
Indirectly part of >>
Also associated with >>

11. Acquired causes :
Secondary ??
Toxins >> lead or zinc poisoning
Drugs >> ethanol , INH, chloromphenicol
Nutritional >> pyredoxine or copper def
Indirectly part of >> myelodysplastic
syndrome .
Also associated with >> Leukemia ,
Lymphoma , Myeloma , RA , Others .

12. 1. pallor
2. dizziness
3. fatigue
4. liver disease
5. enlarged liver or spleen
6. Cardiac arrhythmia
7. Renal failure

13. Blood
↓Hb
Normal or ↓MCV
↑Serum iron
NormalTotal iron binding capacity
↑Ferritin level
↑Transferrin Saturation

14. Anisocytosis ??
Poikilocytosis ??

17. Bone marrow shows erythroid
hyperplasia with a maturation arrest.
excess of 40% of the
developing erythrocytes are ringed
sideroblasts.

19. Prussian Blue stain of RBC in marrow. Shows
ringed sideroblasts.

21. HF
Pericarditis
Formation of liver nodules & scar tissue
DM
Hypothyroidisim
Secondary Hypopitutarisim ??
Skin darkining
Leukemia >> unknown origin sideroblastic
anemia .

22. In severe anemia >> required Blood
Transfusion ++ Desferrioxamine ??
Erythropoietin ??
Moderate to high dose of pyredoxine (Vit
B6)
Toxic >> remove
In the case of isoniazid-induced sideroblastic
anemia >>> the addition of B6 is sufficient to
correct the anemia .
SEVERE CASESTx ??