title: Relative iron deficiency in hereditary spherocytosis.
abstract: Seventy-three patients with hereditary spherocytosis (HS) (58 nonsplenectomized, 15 splenectomized) were studied to evaluate iron status and the adequacy of iron availability for erythropoiesis. Splenectomized patients, who had hemoglobin levels in the normal or upper normal range, had higher levels of serum iron, transferrin saturation, and serum ferritin than normal matched controls and normal zinc protoporphyrin (ZnPP) levels. On the contrary, nonsplenectomized patients presenting with mild to severe anemia had higher red cell ZnPP concentrations than both splenectomized subjects and matched normal controls. ZnPP in nonsplenectomized patients correlated inversely with Hb concentration, mean corpuscular volume (MCV), mean red cell hemoglobin concentration (MCHC), transferrin saturation, and serum iron, and directly with reticulocyte count. At multiple regression analysis only Hb concentration was a significant explanatory variable for high ZnPP. The authors conclude that a number of nonsplenectomized HS patients have relative iron deficiency primarily because of expansion of erythropoiesis caused by anemia.
authors.affiliation: Centro Trasfusionale e di Immunologia dei Trapianti, Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Zanella
authors.forename: G
authors.initials: G
authors.lastname: Barosi
authors.forename: A
authors.initials: A
authors.lastname: Berzuini
authors.forename: M B
authors.initials: MB
authors.lastname: Colombo
authors.forename: S
authors.initials: S
authors.lastname: Quaglini
authors.forename: D
authors.initials: D
authors.lastname: Volpes
authors.forename: G
authors.initials: G
authors.lastname: Sirchia
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004909:Erythrocyte Indices; D005260:Female; D006801:Humans; D007501:Iron / Q000097:blood; D000090463:Iron Deficiencies; D008297:Male; D008875:Middle Aged; D011524:Protoporphyrins / Q000097:blood; D012044:Regression Analysis; D013103:Spherocytosis, Hereditary / Q000097:blood*
chemical_list: D011524:Protoporphyrins; C017803:zinc protoporphyrin; D007501:Iron