Treatment of primary defective iron-reutilization syndrome: revisited

• Published in: Annals of hematology Vol. 79; no. 8; pp. 465 - 468
• Main Authors: BESA, E. C, KIM, P. W, HAURANI, F. I
• Format: Journal Article
• Language: English
• Published: Berlin Springer 01.08.2000; Springer Nature B.V
• Subjects: Aged; Anemia, Iron-Deficiency - drug therapy; Anemia, Iron-Deficiency - metabolism; Anemias. Hemoglobinopathies; Biological and medical sciences; Cohort Studies; Danazol - therapeutic use; Diseases of red blood cells; Estrogen Antagonists - therapeutic use; Female; Hematologic and hematopoietic diseases; Humans; Iron - metabolism; Medical sciences; Middle Aged; Human; Disease; Anemia; Toxicity; Treatment efficiency; Hemopathy; Danazol; Differential diagnostic; Case study; Chronic; Iron ion; Etiology; Negative therapeutic reaction
• ISSN: 0939-5555; 1432-0584
• DOI: 10.1007/s002770000180

We encountered two patients who presented with hypochromic-microcytic anemia and were refractory to iron therapy. The symptoms were suggestive of anemia of chronic disease (ACD); however, there was no evidence of any such disease, either inflammatory or malignant. These patients were reminiscent of patients originally described as having primary defective iron reutilization. The hematologic picture consisted of hypochromic-microcytic anemia, low serum iron, low to normal iron binding capacity, high serum ferritin, and increased bone marrow iron in the absence of ringed sideroblasts. These patients had symptomatic anemia and received danazol (200 mg orally) three times per day to which they responded very well with an increase of approximately 3 g in the hemoglobin concentration over 1 year and amelioration of their symptoms. Danazol was well tolerated and did not cause any virilizing side effects. Doses were lowered in maintenance after 1 year to 200 mg once per week, and responses were sustained up to 36 months of follow-up duration. In the differential diagnosis of hypochromic-microcytic anemia, especially in postmenopausal women, one has to consider this type of treatable anemia when more common types such as iron deficiency, chronic inflammation, malignancy, sideroblastic anemia, or thalassemia have been ruled out.