Treatment based on manifestation and root cause of disease of patients with non-transfusion dependent thalassemia
LEI Yu ZENG Qing CHENG Weimin
Department of Hematology, the First Affiliated Hospital of Guangxi University of Chinese Medicine, Guangxi Zhuang Autonomous Region, Nanning 530023, China
Abstract：Non-transfusion dependent thalassemia is a chronic hemolytic disease. During the acute attack, hemolysis and blood transfusion should be controlled, and iron removal should be paid attention to at ordinary times. After summing up the treatment based on syndrome differentiation of traditional Chinese medicine of doctors of all dynasties and combining the modern medical theory to clarify the relationship between kidney deficiency and blood stasis, modern Chinese medicine scholars take treatment based on syndrome differentiation and refining for the non-transfusion dependent thalassemia patients and put forward the sequential therapy of treating the tip at the acute condition, treating both manifestation and root cause of disease at stable period and further improve the patient′s hemoglobin to reduce the frequency of blood transfusion and iron deposition, so as to achieve the goal of improving the therapeutic effect of traditional Chinese medicine and the quality of life of patients.
雷宇 曾清 程纬民. 非输血依赖型地中海贫血患者的标本论治[J]. 中国医药导报, 2020, 17(5): 133-136.
LEI Yu ZENG Qing CHENG Weimin. Treatment based on manifestation and root cause of disease of patients with non-transfusion dependent thalassemia. 中国医药导报, 2020, 17(5): 133-136.
[1] 庞艳敏，王小超，潘红飞，等.广西地区1110例血红蛋白H病患者的基因型、血液学表型和铁过载的临床研究[J].右江医学，2019，47（5）：336-342.
[2] Weatherall DJ. The definition and epidemiology of non-transfusion-dependent thalassemia [J]. Blood Rev，2012， 26 Suppl 1：S3-S6. Doi：10.1016/S0268-960X（12）70003-6.
[3] 陈郁婷，黄霄天，刘幸，等.铁过载对神经元功能的调控作用研究[J].神经药理学报，2017，7（2）：12-13.
[4] 蒙苗，林晓思，卢小娟，等.铁过载抑制神经细胞Rab8A蛋白表达[J].基因组学与应用生物学，2018，37（4）：1838-1841.
[5] 王璐，张倩，韩冰.无效造血与铁过载[J].基础医学与临床，2014，34（1）：121-124.
[6] Liang Y，Bajoria R，Jiang Y，et al. Prevalence of diabetes mellitus in Chinese children with thalassaemia major [J]. Trop Med Int Health，2017，22（6）：716-724. doi：10.1111/tmi.12876.
[7] 刘赫.线粒体铁代谢与血色病的关系、特征及处理[J].实用糖尿病杂志，2019，15（1）：10-12.
[8] Pasricha SR，Frazer DM，Bowden DK，et al. Transfusion suppresses erythropoiesis and increases hepcidin in adult patients with β-thalassemia major：a longitudinal study [J]. Blood，2013，122（1）：124-133. doi：10.1182/blood-2012-12-471441.
[9] 吴志奎，张新华，方素萍，等.基于“肾藏精生髓”理论治疗地中海贫血[J].中医杂志，2011，52（1）：20-23.
[10] 褚娜利，张新华，吴志奎，等.补肾益髓法治疗血红蛋白H病237例临床研究[J].医学研究杂志，2015，44（5）：48-51.
[11] Huang Y，Lei Y，Liu R，et al. Imbalance of erythropoiesis and iron metabolism in patients with thalassemia [J]. Int J Med Sci，2019，16（2）：302-310. doi：10.7150/ijms.27829.
[12] Chauhan R，Sharma S，Chandra J. What regulates hepcidin in poly-transfused β-thalassemia major：erythroid drive or store drive? [J]. Indian J Pathol Microbiol，2014，57（1）：39-42. doi：10.4103/0377-4929.130891.
[13] Origa R，Cazzola M，Mereu E，et al. Differences in the erythropoiesis-hepcidin-iron store axis between hemoglobin H disease and β-thalassemia intermedia [J]. Haematologica，2015，100（5）：e169-e171. doi：10.3324/haematol.2014.115733.
[14] Kautz L，Jung G，Valore EV，et al. Identification of erythroferrone as an erythroid regulator of iron metabolism [J]. Nat Genet，2014，46（7）：678-684. doi：10.1038/ng.2996.
[15] Dussiot M，Maciel TT，Fricot A，et al. An activin receptor ⅡA ligand trap corrects ineffective erythropoiesis in β-thalassemia [J]. Nat Med，2014，20（4）：398-407. doi：10.1038/nm.3468.
[16] Schmidt PJ，Fleming MD. Modulation of hepcidin as therapy for primary and secondary iron overload disorders：preclinical models and approaches [J]. Hematol Oncol Clin North Am，2014，28（2）：387-401. doi：10.1016/j.hoc.2013.11.004.
[17] 刘容容.铁过载对地中海贫血患者造血功能的损伤和机制研究[J].广西医科大学学报，2019，36（6）：1018-1021.
[18] Anderson ER，Taylor M，Xue X，et al. Intestinal HIF2α promotes tissue-iron accumulation in disorders of iron overload with anemia [J]. Proc Natl Acad Sci USA，2013， 110（50）：E4922-E4930. doi：10.1073/pnas.1314197110.
[19] 王杜娟.葛根素与铁过载相关疾病的研究进展[J].实用临床医学，2019，20（4）：104-106.
[20] 方华珍，丁成华，郑绍勇.活血化瘀药抗肝纤维化的临床体会[J].实用中西医结合临床，2014，14（3）：60-61.
2020, Vol. 17 
京公网安备 11010502046598号