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Definition

A form of sideroblastic anemia that shows a variable hematologic response to pharmacologic doses of pyridoxine. Sideroblastic anemia is characterized by anemia of varying severity, hypochromic peripheral erythrocytes, systemic iron overload secondary to chronic ineffective erythropoiesis, and the presence of bone marrow ringed sideroblasts. Sideroblasts are characterized by iron-loaded mitochondria clustered around the nucleus.

Acronym

SIDBA1

Synonyms

Anemia, sideroblastic, X-linked
ANH1
Hereditary iron-loading anemia
Hereditary sideroblastic anemia
Hypochromic anemia
XLSA