Pituitary iron and volume predict hypogonadism in transfusional iron overload
Pituitary iron and volume predict hypogonadism in transfusional iron overload
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DOI：
10.1002/ajh.22247
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发表时间：
2012-02-01
影响因子：
12.8
通讯作者：
Wood, John C.
中科院分区：
文献类型：
--
作者：
Noetzli, Leila J.;Panigrahy, Ashok;Wood, John C.
性腺功能减退症是输血依赖性贫血（例如重型地中海贫血）患者最常见的发病率。我们使用磁共振成像 (MRI) 测量垂体 R2（铁）和体积，以确定这些患者在什么年龄出现垂体铁过载和体积减少。我们招募了 56 名患者（47 名重型地中海贫血患者、5 名慢性输血中间型地中海贫血患者和 4 名 Blackfan-Diamond 综合征患者）进行垂体 MRI 测量，以测量垂体 R2 和体积。性腺功能减退症在临床上是根据第二性征出现的时间或性激素替代治疗的需要来定义的。输血性铁超负荷的患者在生命的最初十年开始出现垂体铁超负荷；然而，直到生命的第二个十年才观察到临床上显着的体积损失。严重的垂体铁沉积（Z > 5）和体积减少（Z < -2.5）是性腺功能减退的独立预测因素。通过 MRI 检测，垂体 R2 与血清铁蛋白以及肝脏、胰腺和心脏铁沉积显着相关。对数胰腺 R2* 是垂体铁的最佳单一预测因子，接收算子特征曲线下面积为 0.88，但对数心脏 R2* 和铁蛋白在多变量回归中保留，组合 r2 为 0.71。垂体铁超负荷和容量减少是性腺功能减退症的独立预测因素。许多中度至重度垂体铁超负荷的患者保留了正常的腺体体积和功能，代表了潜在的治疗窗口。保留腺体体积的性腺功能减退患者亚群也可以解释强化螯合治疗后观察到的垂体功能的改善。是。 J.赫马托尔。 2011。(C) 2011 Wiley 期刊公司。
Hypogonadism is the most common morbidity in patients with transfusion-dependent anemias such as thalassemia major. We used magnetic resonance imaging (MRI) to measure pituitary R2 (iron) and volume to determine at what age these patients develop pituitary iron overload and volume loss. We recruited 56 patients (47 with thalassemia major, five with chronically transfused thalassemia intermedia and four with Blackfan-Diamond syndrome) to have pituitary MRIs to measure pituitary R2 and volume. Hypogonadism was defined clinically based on the timing of secondary sexual characteristics or the need for sex hormone replacement therapy. Patients with transfusional iron overload begin to develop pituitary iron overload in the first decade of life; however, clinically significant volume loss was not observed until the second decade of life. Severe pituitary iron deposition (Z > 5) and volume loss (Z < -2.5) were independently predictive of hypogonadism. Pituitary R2 correlated significantly with serum ferritin as well as liver, pancreatic, and cardiac iron deposition by MRI. Log pancreas R2* was the best single predictor for pituitary iron, with an area under the receiving operator characteristic curve of 0.88, but log cardiac R2* and ferritin were retained on multivariate regression with a combined r2 of 0.71. Pituitary iron overload and volume loss were independently predictive of hypogonadism. Many patients with moderate-to-severe pituitary iron overload retained normal gland volume and function, representing a potential therapeutic window. The subset of hypogonadal patients having preserved gland volumes may also explain improvements in pituitary function observed following intensive chelation therapy. Am. J. Hematol. 2011. (C) 2011 Wiley Periodicals, Inc.