Effectiveness of erythrocytapheresis in idiopathic hemochromatosis. Report of 14 cases. 1989

D Conte, and C Mandelli, and M Cesana, and R Ferrini, and M Marconi, and A Bianchi
Institute of Medical Pathology III, University of Milan, Italy.

Thirteen men and one woman (mean age 48.8 yr +/- 6.9, range 36-63) with idiopathic hemochromatosis were treated by erythrocytapheresis. Iron depletion followed 9.60 months treatment (median 24), with 21-203 erythrocytaphereses (mean 93 +/- 61) and total iron removal of 4.2-40.6 g (mean 19 +/- 11.9). Trasferrin saturation decreased from 90 +/- 8.7% to 17 +/- 10.6% and serum ferritin from 3164 micrograms/L +/- 1488 to 60.5 micrograms/L +/- 77.5, and liver iron content normalized in all cases. Initial serum ferritin in the patients who were iron-depleted at 18 months (50%, cumulative percentage) was significantly lower than in those still iron loaded at that time (2280 micrograms/L +/- 940 vs 4049 micrograms/L +/- 1444, p less than 0.02). Clinical improvement was noted in all cases with about a 30% decrease in insulin requirement in most diabetics. Thus erythrocytapheresis appears to be effective and safe in obtaining iron depletion in idiopathic hemochromatosis.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D001781 Blood Component Removal Any procedure in which blood is withdrawn from a donor, a portion is separated and retained, at the same time the remainder is returned to the donor. Apheresis,Pheresis,Aphereses,Blood Component Removals,Phereses,Removal, Blood Component
D005260 Female Females
D005293 Ferritins Iron-containing proteins that are widely distributed in animals, plants, and microorganisms. Their major function is to store IRON in a nontoxic bioavailable form. Each ferritin molecule consists of ferric iron in a hollow protein shell (APOFERRITINS) made of 24 subunits of various sequences depending on the species and tissue types. Basic Isoferritin,Ferritin,Isoferritin,Isoferritin, Basic
D006432 Hemochromatosis A disorder of iron metabolism characterized by a triad of HEMOSIDEROSIS; LIVER CIRRHOSIS; and DIABETES MELLITUS. It is caused by massive iron deposits in parenchymal cells that may develop after a prolonged increase of iron absorption. (Jablonski's Dictionary of Syndromes & Eponymic Diseases, 2d ed) Diabetes, Bronze,Bronze Diabetes,Bronzed Cirrhosis,Familial Hemochromatosis,Genetic Hemochromatosis,Haemochromatosis,Hemochromatoses,Iron Storage Disorder,Pigmentary Cirrhosis,Primary Hemochromatosis,Troisier-Hanot-Chauffard Syndrome,Von Recklenhausen-Applebaum Disease,Bronzed Cirrhoses,Cirrhoses, Bronzed,Cirrhoses, Pigmentary,Cirrhosis, Bronzed,Cirrhosis, Pigmentary,Disease, Von Recklenhausen-Applebaum,Diseases, Von Recklenhausen-Applebaum,Disorder, Iron Storage,Disorders, Iron Storage,Familial Hemochromatoses,Genetic Hemochromatoses,Haemochromatoses,Hemochromatose,Hemochromatoses, Familial,Hemochromatoses, Genetic,Hemochromatosis, Familial,Hemochromatosis, Genetic,Iron Storage Disorders,Pigmentary Cirrhoses,Recklenhausen-Applebaum Disease, Von,Recklenhausen-Applebaum Diseases, Von,Storage Disorder, Iron,Storage Disorders, Iron,Syndrome, Troisier-Hanot-Chauffard,Syndromes, Troisier-Hanot-Chauffard,Troisier Hanot Chauffard Syndrome,Troisier-Hanot-Chauffard Syndromes,Von Recklenhausen Applebaum Disease,Von Recklenhausen-Applebaum Diseases
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D014168 Transferrin An iron-binding beta1-globulin that is synthesized in the LIVER and secreted into the blood. It plays a central role in the transport of IRON throughout the circulation. A variety of transferrin isoforms exist in humans, including some that are considered markers for specific disease states. Siderophilin,Isotransferrin,Monoferric Transferrins,Serotransferrin,Transferrin B,Transferrin C,beta 2-Transferrin,beta-1 Metal-Binding Globulin,tau-Transferrin,Globulin, beta-1 Metal-Binding,Metal-Binding Globulin, beta-1,Transferrins, Monoferric,beta 1 Metal Binding Globulin,beta 2 Transferrin,tau Transferrin
D017707 Erythrocyte Transfusion The transfer of erythrocytes from a donor to a recipient or reinfusion to the donor. Red Blood Cell Transfusion,Red Blood Cell Transfusions,Transfusion, Red Blood Cell,Transfusions, Red Blood Cell,Erythrocyte Transfusions,Transfusion, Erythrocyte,Transfusions, Erythrocyte

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