title: [Histological picture of liver disease in thalassemia intermedia].
abstract: Thalassemia Intermedia (TI) is a clinical definition in use for a spectrum of clinical conditions ranging in severity from the asymptomatic carrier status to the transfusion-dependent status. The histological lesions of the liver in patients affected by TI has not seen well characterized yet. The aim of this study was to define the histological picture of liver disease in TI. To this end we studied our pool of 22 Thalassemia Intermedia patients who underwent liver biopsy; none of them had blood transfusion. We took into consideration fibrosis according to Ishak's staging and iron overload according to Sciot's grading. Moreover, we determined the hepatic iron content by atomic absorption spectroscopy. Our results have shown that, in all patients studied, the histological picture was characterized by the increase in liver iron stores. By Perls's stain haemosiderin appeared to be stored mainly in hepatocytes in the majority of cases. Inspite of the large amounts of liver iron in these patients, fibrosis was absent or mild. We also noted the presence of iron-free-foci, in part of the patients. Our data show that the histological and histochemical picture of liver disease in Thalassemia Intermedia is very similar to that found in Thalassemia Major. The finding of iron-free-foci could induce to consider a follow up of these patients, even if asymptomatic.
authors.affiliation: Dipartimento di Citomorfologia, Università di Cagliari. mgmancosu@tiscalinet.it
authors.forename: M G
authors.initials: MG
authors.lastname: Mancosu
authors.forename: R
authors.initials: R
authors.lastname: Galanello
authors.forename: R
authors.initials: R
authors.lastname: Ambu
authors.forename: S
authors.initials: S
authors.lastname: De Virgiliis
authors.forename: N
authors.initials: N
authors.lastname: Giagu
authors.forename: A
authors.initials: A
authors.lastname: Cao
authors.forename: G
authors.initials: G
authors.lastname: Faa
mesh_terms: D000293:Adolescent; D000328:Adult; D001707:Biopsy, Needle; D005260:Female; D006485:Hemosiderin / Q000032:analysis; D022781:Hepatocytes / Q000737:chemistry / Q000648:ultrastructure; D006801:Humans; D007501:Iron / Q000032:analysis; D019190:Iron Overload / Q000209:etiology / Q000473:pathology; D007728:Kupffer Cells / Q000737:chemistry / Q000648:ultrastructure; D008099:Liver / Q000737:chemistry; D008103:Liver Cirrhosis / Q000209:etiology / Q000473:pathology; D008107:Liver Diseases / Q000209:etiology / Q000473:pathology*; D008297:Male; D012720:Severity of Illness Index; D017086:beta-Thalassemia / Q000150:complications*
chemical_list: D006485:Hemosiderin; D007501:Iron