中间型地中海贫血青春期前大量输血：对生长、脾功能和内分泌参数持续产生积极影响的病例报告
Prepubertal Hypertransfusion in Thalassemia Intermedia: A Case Report of Sustained Positive Effects on Growth, Splenic Function and Endocrine Parameters.
作者信息
Tony Surekha, Daar Shahina, Zachariah Mathew, Wali Yasser
出版信息
Oman Med J. 2012 Nov;27(6):e012. doi: 10.5001/omj.2012.125.
Abstract
We report a known case of thalassemia intermedia (Hb Dhofar) who in spite of mild thalassemic facies, attained his normal genetic height, pubertal maturity and improved self-image with minimal splenomegaly through a hypertransfusion/chelation regimen that was started just before puberty and maintained for 5 years. As there are no clear guidelines in the management of patients with thalassemia intermedia, the option of hypertransfusion/chelation during the pubertal growth spurt may alleviate some of the complications associated with thalassemia intermedia.
摘要
我们报告了一例中间型地中海贫血（Hb 佐法尔）的已知病例，尽管有轻度地中海贫血面容，但通过在青春期前开始并维持 5 年的强化输血/螯合疗法，该患者达到了正常的遗传身高、青春期成熟度，并改善了自我形象，且脾肿大程度最小。由于中间型地中海贫血患者的管理尚无明确指南，在青春期生长突增期间进行强化输血/螯合疗法可能会减轻一些与中间型地中海贫血相关的并发症。
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