Sideroblastic anemia

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Sideroblastic anemia is caused by the abnormal production of red blood cells as part of myelodysplastic syndrome, which can evolve into hematological malignancies (especially acute myelogenous leukemia).

1 Causes
2 Diagnosis
3 Laboratory findings
4 Treatment
5 See also
6 End Notes
7 External links

[edit] Causes

The common feature of these causes is a failure to completely form heme - whose biosynthesis takes place partly in the mitochondrion. This leads to deposits of iron in the mitochondria that form a ring around the nucleus of the developling red blood cell. Sometimes the disorder represents a stage in evolution of a generalized bone marrow disorder that may ultimately terminate in acute leukemia.

Toxins: lead or zinc poisoning
Drug-induced: ethanol, isoniazid, chloramphenicol, cycloserine
Nutritional: pyridoxine or copper deficiency
Genetic: ALA synthase deficiency (X-linked)

[edit] Diagnosis

Ringed sideroblasts are seen in the bone marrow.

[edit] Laboratory findings

Increased ferritin levels
Decreased total iron-binding capacity
Hematocrit of about 20-30%
Serum Iron: High
High transferrin saturation
The mean corpuscular volume or MCV is usually normal or slightly increased; although it may occasionally be low, leading to confusion with iron deficiency.^[1]
With lead poisoning, see coarse basophilic stippling of red blood cells on peripheral blood smear
Specific test: Prussian Blue stain of RBC in marrow. Shows ringed sideroblasts.

[edit] Treatment

Occasionally, the anemia is so severe that support with transfusion is required. These patients usually do not respond to erythropoietin therapy.^[2]

[edit] See also

[edit] End Notes

↑ "Sideroblastic Anemia" Tierney/McPhee/Papadakis Current Medical Diagnosis and Treatment. 45th Ed. McGraw-Hill. ISBN: 0-07-145410-1.
↑ "Sideroblastic Anemia" Tierney/McPhee/Papadakis Current Medical Diagnosis and Treatment. 45th Ed. McGraw-Hill. ISBN: 0-07-145410-1.