Sideroblastic Anemia

Description
Sideroblastic anemias nucleated eryhtroid
precursors found in the bone marrow happens to be
ringed sideroblasts
Came from many etiology
Siderocyte are erythrocytesthat contains iron
cytoplasmic granules, if it happens on a normoblast,
called as sideroblast
Siderocytes usually not present in human blood, can
be stained with Prussian blue reaction and
romanowsky dyes.
Sideroblastic anemia is diagnosed when 15% or
more of marrow erythroblasts are ring sideroblasts
Types
Hereditary
Rare X-linked disease
Defective ALA synthetase
enzyme activity
ALA synthetase synthesize
aminolevulinin acid required
for hem synthesis
Acquired
1.Primary idiopathic acquired
Sideroblastic anemia
2.Secondary acquired
sideroblastic anemia causes
1.Drugs, chemicals and toxins
(alcohol, INH, lead, vit D def)
2.Myelofibrosis, acute leukemia,
myeloma and other
hematological disorders
3.Carcinoma, RA

Sign and Symptoms
General symptoms of anemia, including malaise, fatigue, and dyspnea on
exertion
Incoordination (cerebellar symptoms)
Failure of growth
Diarrhea (malabsorption)
Polyuria, blindness, deafness (associated with DIDMOAD syndrome)
History of exposure to cold for prolonged periods
Family history of mitochondrial disease and anemia
Medication history of antibiotics, antituberculous agents, chelators, or
chemotherapy

Lab Works
CBC and peripheral smear: shows anemia signs, MCV (mean
corpuscular volume) low, sideroblast and siderocytes can be seen in
peripheral blood
Iron studies: increased iron level with decreased total iron bindng
capacity, low ferritin levels
Bone marrow aspiration biopsy shows more than 15% ring
sideroblasts
Management
Removal of toxic agents
Administration of pyridoxine, thiamine or folic acid (vitamin B
therapy)
Transfutsion
Bone marrow or liver transplant
Antidotes if iron overdose
Deferoxamine
Deferasirox
References
Sideroblast [image].[cited 8 5 2014] available from:
http://imagebank.hematology.org/Content%5C506%5C3193%5C3193
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Siderocyte [image].[cited 8 5 2014] available from:
http://imagebank.hematology.org/Content%5C609%5C3524%5C3524
_full.JPG
Sideroblastic anemia [website].[cited 8 5 2014] available from:
http://emedicine.medscape.com/article/1389794-overview#a0104