Increased prevalence of iron‐overload associated endocrinopathy in thalassaemia versus sickle‐cell disease

• Published in: British journal of haematology Vol. 135; no. 4; pp. 574 - 582
• Main Authors: Fung, Ellen B., Harmatz, Paul R., Lee, Phillip D.K., Milet, Meredith, Bellevue, Rita, Jeng, Michael R., Kalinyak, Karen A., Hudes, Mark, Bhatia, Suruchi, Vichinsky, Elliott P.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.11.2006; Blackwell
• Subjects: Adult; Anemia, Sickle Cell - complications; Anemia, Sickle Cell - therapy; Anemias. Hemoglobinopathies; Anthropometry; Biological and medical sciences; Diabetes Mellitus, Type 2 - etiology; Diseases of red blood cells; Endocrine System Diseases - etiology; endocrinopathy; Female; Growth Disorders - etiology; Hematologic and hematopoietic diseases; Humans; Hypogonadism - etiology; Hypothyroidism - etiology; iron overload; Iron Overload - complications; Male; Medical sciences; Middle Aged; Prospective Studies; sickle‐cell disease; thalassaemia; Thalassemia - complications; Thalassemia - therapy; Transfusion Reaction; transfusion therapy; Endocrinopathy; transfusion therapy; Hemoglobinopathy; iron overload; Sickle cell anemia; Prevalence; Transfusion; thalassaemia; Hematology; Thalassemia; Hemopathy; Iron; Epidemiology; Genetic disease; Hemolytic anemia; Treatment; Overload; sickle-cell disease; Comparative study
• ISSN: 0007-1048; 1365-2141
• DOI: 10.1111/j.1365-2141.2006.06332.x

Summary 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.