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Microcytic anemia with iron malabsorption: An inherited disorder of iron metabolism

✍ Scribed by Hartman, Kip R.; Barker, James A.

Publisher: John Wiley and Sons
Year: 1996
Tongue: English
Weight: 601 KB
Volume: 51
Category: Article
ISSN: 0361-8609
DOI: 10.1002/(sici)1096-8652(199604)51:4<269::aid-ajh4>3.0.co;2-u

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✦ Synopsis

Two siblings were identified with severe hypoproliferative mlcrocytlc anemia and Iron malabsorption, In the absence of any gastrointestinal disorder or blood loss. These children had severe mlcrocytosls (MCV 48 fl, hemoglobin 7.5 gldl) with decreased serum iron, elevated serum TIBC, and decreased serum ferritln, despite prolonged treatment with oral Iron. An Iron challenge study with an oral dose of 2 mglkg elemental Iron as ferrous sulfate documented iron malabsorption. After treatment with intravenous iron dextran, there was an absence of the expected retlculocytosls and only a partial correction of the hemoglobin, hematocrlt, and mlcrocytosls. The bone marrow was hypocellular with abnormal Iron Incorporation Into erythrold precursor cells. This appears to be a rare form of Inherited anemla characterized by Iron malabsorption and disordered Iron metabolism that only partially corrects after the administration of parenteral Iron. These features resemble those found In the microcytic mouse (mWmk), which also has severe microcytic anemia and iron malabsorption that partially responds to parenteral Iron.

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