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3 out of 8

Sideroblastic anameia is seen in?

A Myelofibrosis
B Myeloma

D All of the above

Ans. D

All of the above

1. Pathophysiology of sideroblastic anemia

a. Caused by disorder in the synthesis of the heme moiety of hemoglobin they are characterized by presence of trapped iron in the mitochondria of nucleated RBC.

b. Most of the enzymes for protoporphyrin synthesis are located in the nucleated RBC mitochondria.

c. Derangements in these pathways cause iron accumulation in the perinuclear mitochondria, which renders this anemia its characteristic morphologic finding of ringed sideroblasts.

d. RBC Population is hypochromic and microcytic. It leads to ineffective erythropoiesis

e. Defect: There is defect in incorporating Fe into Hb within RBC

f. P/s. microcytic hypochromic or dimorphic.

2. Important Points::

a. Sideroblast - Erythroblast with Prussian blue positive iron granules in cytoplasm.

b. Siderocytes - Mature RBC with Prussian blue positive iron granules in cytoplasm.

- Granules also positive for iron with Romanovsky dye

- Granules appears as basophilic granules called as Pappenheimer bodies.

c. Ring Sideroblast – Sideroblast in which iron granules forms partial / complete ring around nucleus (Ring is made up of mitochondria with iron granules).

Causes of Sideroblastic Anemia

1. Hereditary

a. X-linked

b. Mitochondrial

c. Autosomal dominant

2. Acquired

a. Primary : MDS

b. Secondary

3. Hematological:

a. Myelofibrosis

b. Polycythemia vera

c. Acute leukemia

d. Myeloma

e. Lymphoma

f. Hemolytic anemia

4. Other Diseases:

a. Rheumatoid arthritis

b. Myxoedema

5. Drugs chemicals and toxins

a. Isoniazid, Penicillamine

b. Alcohol

c. pyridoxine deficiency

d. Lead poisoning.

Extra Edge:

Pearson syndrome occurs in congenital sideroblastic anemia, there is pancytopenia with macrocytosis.