The primary defective iron reutilization syndrome involves a microcytic, hypochromic anemia resistant to iron replacement occurring in a patient with no evidence of anemia of chronic disease or other hematologic disorder.

Patient characteristics:

(1) typically post-menopausal women

(2) responds to testosterone.


Criteria for primary defective iron reutilization:

(1) hypochromic microcytic anemia

(1a) hemoglobin < 120 g/L

(1b) MCV < 82 femtoliters

(1c) MCH < 27 pg/cell

(2) decreased serum iron studies

(2a) reduced serum iron

(2b) total iron binding capacity (TIBC) low or normal

(2c) iron saturation < 20%

(3) increased iron stores

(3a) serum ferritin levels > 30 µg/L

(3b) abundant bone marrow iron without ringed sideroblasts

(4) normal hemoglobin electropheresis (with hemoglobin A2 < 3.5%) with no evidence of thalassemia

(5) normal lead level

(6) absence of a primary inflammatory or malignant disease

(7) no response to prolonged (3 –6 months) oral iron replacement



• The reference gives the iron in mol/L. The SIU is µmol/L.

• The reference gives a reduced serum iron as < 5.4 µmol/L. I have used < 9 µmol/L in the spreadsheet.

• The reference gives serum ferritin in g/L. The SIU units are µg/L.


Findings on erythrokinetics are similar to that seen in anemia of chronic disease:

(1) normal plasma iron turnover

(2) normal red blood cell iron utilization

(3) poor red blood cell iron reutilization

(4) poor iron absorption


Treatment: Patients have responded with danazol, an isoxazole derivative of 17-alpha ethinyl testosterone, given in a dose of 200 mg once a week.

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