Ang Ai Leen, Tzoulis Ploutarchos, Prescott Emma, Davis Bernard A, Barnard Maria, Shah Farrukh T
Department of Haematology, The Whittington Hospital, London, UK; Department of Haematology, Singapore General Hospital, Singapore.
Eur J Haematol. 2014 Mar;92(3):229-36. doi: 10.1111/ejh.12224.
Endocrinopathies are common complications of transfusional hemosiderosis among patients with β thalassemia major (TM). Previous studies had shown associations between some endocrinopathies and iron overload of the myocardium, liver and/or endocrine organs as assessed by MRI techniques. This retrospective analysis of 92 patients with TM (median age 36 yr) from a tertiary adult thalassemia unit in UK aimed to determine independent risk factors associated with endocrinopathies among these patients. Unlike previous studies, longitudinal data on routine measurements of iron load [worst myocardial and liver T2* values since 1999, worst LIC by MRI-R2 since 2008 and average 10-yr serum ferritin (SF)] up to April 2010 together with demographic features and age of initiating chelation were analyzed for associations with endocrinopathies. The most common endocrinopathies in this cohort were hypogonadism (67%) and diabetes mellitus (DM) (41%), and these were independently associated with myocardial T2* <20 ms (P < 0.001 and P = 0.008, respectively) and increased age (P = 0.002 and P = 0.016, respectively). DM and hypogonadism were independently associated with average SF >1250 μg/L (P = 0.003) and >2000 μg/L (P = 0.047), respectively. DM was also associated with initial detection of abnormal myocardial T2* at an older age (30 yr vs. 24 yr, P = 0.039). An abnormal myocardial T2* may therefore portend the development of DM and hypogonadism in patients with TM.
内分泌病是重型β地中海贫血（TM）患者输血性含铁血黄素沉着症的常见并发症。既往研究表明，通过MRI技术评估，某些内分泌病与心肌、肝脏和/或内分泌器官的铁过载之间存在关联。这项对英国一家三级成人地中海贫血治疗中心的92例TM患者（中位年龄36岁）进行的回顾性分析，旨在确定这些患者中与内分泌病相关的独立危险因素。与既往研究不同的是，分析了截至2010年4月的铁负荷常规测量的纵向数据[1999年以来最差的心肌和肝脏T2值、2008年以来MRI-R2测量的最差肝脏铁浓度（LIC）以及10年血清铁蛋白（SF）平均值]，以及人口统计学特征和开始螯合治疗的年龄，以寻找与内分泌病的关联。该队列中最常见的内分泌病是性腺功能减退（67%）和糖尿病（DM）（41%），它们分别与心肌T2<20 ms独立相关（P<0.001和P=0.008）以及年龄增加独立相关（P=0.002和P=0.016）。DM和性腺功能减退分别与平均SF>1250μg/L（P=0.003）和>2000μg/L（P=0.047）独立相关。DM还与在较高年龄（30岁对24岁，P=0.039）首次检测到异常心肌T2有关。因此，异常心肌T2可能预示着TM患者发生DM和性腺功能减退。
