SIDEROBLASTIC ANAEMIA

• Sideroblastic anaemia is a group of disorder in which erythroid precursor in the bone marrow show characteristic ‘ringed sideroblastic’.
• Siderocytes are erythrocytes & sideroblasts are normoblasts.
• Siderocytes contains granules of non- heme iron.
• Granules are positive with Prussian blue reaction & Romanowsky dyes called Pappenheimer bodies.
• Sideroblasts nucleated red cells.

TYPES OF SIDEROBLAST ANAEMIAS-

1. Herediatry sideroblastic anaemia

• Rare X- linked disorder associated with defective enzyme aminolevulinic acid (ALA) synthetase for haem synthesis.

 2. Acquired sideroblastic anaemia-

1. Primary-
2. Secondary-
3. Alcohol & lead. (Sideroblastic anaemia is seen in chronic poisoning of lead)

Lab findings-

• Blood picture- hypochromic, microcytic.
• MCV, MCH, MCHC decrease in hereditary type.
• MCV increase in acquired type.
• Bone marrow examination- macronormoblastic erythropoiesis.
• Marrow iron stores increase.
• Pathognomonic ring sideroblasts present serum ferritin increase.
• Serum iron increase.
• Iron deposition increase.

TREATMENT-

• No definite treatment.
• Pyridoxine dose.
• Blood transfusion can be used for treatment.