title: Increased prevalence of iron-overload associated endocrinopathy in thalassaemia versus sickle-cell disease.
abstract: Iron-overload associated endocrinopathy is the most frequently reported complication of chronic transfusion therapy in patients with thalassaemia (Thal). This study compared iron-overloaded subjects with Thal (n = 142; 54%M; age 25.8 +/- 8.1 years) and transfused sickle-cell disease (Tx-SCD; n = 199; 43%M, 24.9 +/- 13.2 years) to non-transfused SCD subjects (non-Tx-SCD; n = 64, 50%M, 25.3 +/- 11.3 years), to explore whether the underlying haemoglobinopathy influences the development of endocrinopathy. Subjects were recruited from 31 centres in the USA, Canada and the UK. Subjects with Thal had more evidence of diabetes (13% vs. 2%, P < 0.001), hypogonadism (40% vs. 4%, P < 0.001), hypothyroidism (10% vs. 2%, P = <0.001) and growth failure (33% vs. 7%, P < 0.001), versus Tx-SCD. Fifty-six per cent of Thal had more than one endocrinopathy compared with only 13% of Tx-SCD (P < 0.001). In contrast, Tx-SCD was not different from non-Tx-SCD. Multivariate analysis indicated that endocrinopathy was more likely in Thal than SCD [Odds Ratio (OR) = 9.4, P < 0.001], with duration of chronic transfusion a significant predictor (OR = 1.4 per 10 years of transfusion, P = 0.04). Despite iron overload, endocrinopathy was not increased in Tx-SCD versus non-Tx-SCD, suggesting that the underlying disease may modulate iron-related endocrine injury. However, because transfusion duration remained a significant predictor of endocrinopathy, these data should be confirmed in SCD subjects that have been chronically transfused for longer periods of time.
authors.affiliation: Department of Hematology, Children's Hospital and Research Centre, Oakland, CA, USA. efung@mail.cho.org
authors.forename: Ellen B
authors.initials: EB
authors.lastname: Fung
authors.forename: Paul R
authors.initials: PR
authors.lastname: Harmatz
authors.forename: Phillip D K
authors.initials: PD
authors.lastname: Lee
authors.forename: Meredith
authors.initials: M
authors.lastname: Milet
authors.forename: Rita
authors.initials: R
authors.lastname: Bellevue
authors.forename: Michael R
authors.initials: MR
authors.lastname: Jeng
authors.forename: Karen A
authors.initials: KA
authors.lastname: Kalinyak
authors.forename: Mark
authors.initials: M
authors.lastname: Hudes
authors.forename: Suruchi
authors.initials: S
authors.lastname: Bhatia
authors.forename: Elliott P
authors.initials: EP
authors.lastname: Vichinsky
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000628:therapy; D000886:Anthropometry; D003924:Diabetes Mellitus, Type 2 / Q000209:etiology; D004700:Endocrine System Diseases / Q000209:etiology*; D005260:Female; D006130:Growth Disorders / Q000209:etiology; D006801:Humans; D007006:Hypogonadism / Q000209:etiology; D007037:Hypothyroidism / Q000209:etiology; D019190:Iron Overload / Q000150:complications*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D013789:Thalassemia / Q000150:complications* / Q000628:therapy; D065227:Transfusion Reaction