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2
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Genotype-phenotype relationship of patients with β-thalassemia taking hydroxyurea: a 13-year experience in Iran.β-地中海贫血患者服用羟基脲的基因型-表型关系：伊朗 13 年的经验。
Int J Hematol. 2012 Jan;95(1):51-6. doi: 10.1007/s12185-011-0985-6. Epub 2011 Dec 20.
2
Optimal management of β thalassaemia intermedia.β 中间型地中海贫血的最佳管理。
Br J Haematol. 2011 Mar;152(5):512-23. doi: 10.1111/j.1365-2141.2010.08486.x. Epub 2011 Jan 20.
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The natural history of thalassemia intermedia.中间型地中海贫血的自然史。
Ann N Y Acad Sci. 2010 Aug;1202:214-20. doi: 10.1111/j.1749-6632.2010.05550.x.
4
Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study.地中海贫血中间型管理实践概述，旨在降低流行地区的并发症发生率：OPTIMAL CARE 研究。
Blood. 2010 Mar 11;115(10):1886-92. doi: 10.1182/blood-2009-09-243154. Epub 2009 Dec 23.
5
Relation between iron-overload indices, cardiac echo-Doppler, and biochemical markers in thalassemia intermedia.中间型地中海贫血中铁过载指标、心脏超声多普勒与生化标志物之间的关系。
Am J Cardiol. 2008 Aug 1;102(3):363-7. doi: 10.1016/j.amjcard.2008.03.066. Epub 2008 May 29.
6
Haematological and clinical features of beta-thalassaemia associated with Hb Dhofar.与血红蛋白D霍法尔相关的β地中海贫血的血液学和临床特征。
Eur J Haematol. 2008 Jan;80(1):67-70. doi: 10.1111/j.1600-0609.2007.00989.x.
7
Prevalence of thromboembolic events among 8,860 patients with thalassaemia major and intermedia in the Mediterranean area and Iran.地中海地区和伊朗8860例重型和中间型地中海贫血患者血栓栓塞事件的患病率。
Thromb Haemost. 2006 Oct;96(4):488-91.
8
Thalassemia intermedia: revisited.中间型地中海贫血：再探讨
Blood Cells Mol Dis. 2006 Jul-Aug;37(1):12-20. doi: 10.1016/j.bcmd.2006.04.005. Epub 2006 Jun 5.
9
Thalassemia heart disease: a comparative evaluation of thalassemia major and thalassemia intermedia.地中海贫血性心脏病：重型与中间型地中海贫血的比较评估
Chest. 2005 May;127(5):1523-30. doi: 10.1378/chest.127.5.1523.
10
Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia.中间型地中海贫血脾切除患者的静脉血栓栓塞和高凝状态
Br J Haematol. 2000 Nov;111(2):467-73. doi: 10.1046/j.1365-2141.2000.02376.x.

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.

DOI:10.5001/omj.2012.125
PMID:28804576
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5547735/
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。