非输血依赖型地中海贫血患者的标本论治
[摘要] 非输血依赖型地中海贫血是一种慢性溶血性疾病，急性发作期间需要控制溶血和输血，平时则要注意祛铁治疗。在总结历代医家中医辨证论治上，同时结合现代医学理论理清肾虚以及瘀血的关系后，现代中医学者对非输血依赖型地中海贫血患者进行中医辨证凝炼，并提出急则治其标、稳定期标本兼治的序贯治疗方法，进一步提高患者血色素，从而减少输血频率以及铁沉积，达到显著提高中医治疗效果、改善患者生存质量的目的。
[关键词] 地中海贫血;标本兼治;铁过载;肝纤维化
[中图分类号] R556 [文献标识码] A [文章编号] 1673-7210(2020)02(b)-0133-04
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.
[Key words] Thalassemia; Treating both manifestation and root cause of disease; Iron overload; Liver fibrosis
地中海貧血是因为人体内调控珠蛋白合成的基因发生功能缺陷，导致体内珠蛋白平衡破坏，从而引起溶血性贫血。目前该病在世界上主要流行于地中海流域以及东南亚区域，而在我国的广西、广东、海南、湖南等南方各省都有一定数量的携带者以及发病人群[1]。根据患者发病症状的严重程度，地中海贫血可分为轻型地中海贫血(地中海贫血携带者)、中间型地中海贫血非输血依赖型地中海贫血、重型地中海贫血(输血依赖型)。地中海贫血携带者仅仅有轻度贫血而没有明显临床症状，而重型地中海贫血患者往往在2～3岁幼童时期就出现重度贫血，该类患者一般每个月需要进行输血，从而发生输血依赖性铁过载。如果患者没能及时得到输血或者输血不规律则会引发机体发育不良(引起严重贫血)，同时会伴随着肝脏和脾脏肿大。中间型地中海贫血临床症状严重程度介于两者之间[2]。查阅相关中医经典，根据目前对地中海贫血的临床症状描述所见，将该病归属于“血证”“血虚”“虚劳”“童子劳”“虚黄”“积聚”等范畴。本病与禀赋薄弱、体质不强密切相关。《灵枢·经脉》说：“人始生，先成精。”父精母血乃构成胚胎发育的初始物质。父母体虚、遗传缺陷、胎中失养、孕育不足是造成禀赋薄弱的主要原因。就本病而言，正因父或母患有此症，才孕育出地中海贫血的胎儿。
1 常见的地中海贫血类型与临床症状
目前临床上常见的中间型地中海贫血有三大类：β-地中海贫血杂合子、HbE/β-双重杂合子地中海贫血和α-地中海贫血。中间型地中海贫血的贫血程度较重型轻，由于此类患者贫血，血红蛋白波动在60～100 g/L，机体携氧和抵抗力比正常人要偏差，同时机体反复慢性溶血，因此容易继发一系列急慢性病变。, 百拇医药(雷宇 曾清 程纬民)
[关键词] 地中海贫血;标本兼治;铁过载;肝纤维化
[中图分类号] R556 [文献标识码] A [文章编号] 1673-7210(2020)02(b)-0133-04
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.
[Key words] Thalassemia; Treating both manifestation and root cause of disease; Iron overload; Liver fibrosis
地中海貧血是因为人体内调控珠蛋白合成的基因发生功能缺陷，导致体内珠蛋白平衡破坏，从而引起溶血性贫血。目前该病在世界上主要流行于地中海流域以及东南亚区域，而在我国的广西、广东、海南、湖南等南方各省都有一定数量的携带者以及发病人群[1]。根据患者发病症状的严重程度，地中海贫血可分为轻型地中海贫血(地中海贫血携带者)、中间型地中海贫血非输血依赖型地中海贫血、重型地中海贫血(输血依赖型)。地中海贫血携带者仅仅有轻度贫血而没有明显临床症状，而重型地中海贫血患者往往在2～3岁幼童时期就出现重度贫血，该类患者一般每个月需要进行输血，从而发生输血依赖性铁过载。如果患者没能及时得到输血或者输血不规律则会引发机体发育不良(引起严重贫血)，同时会伴随着肝脏和脾脏肿大。中间型地中海贫血临床症状严重程度介于两者之间[2]。查阅相关中医经典，根据目前对地中海贫血的临床症状描述所见，将该病归属于“血证”“血虚”“虚劳”“童子劳”“虚黄”“积聚”等范畴。本病与禀赋薄弱、体质不强密切相关。《灵枢·经脉》说：“人始生，先成精。”父精母血乃构成胚胎发育的初始物质。父母体虚、遗传缺陷、胎中失养、孕育不足是造成禀赋薄弱的主要原因。就本病而言，正因父或母患有此症，才孕育出地中海贫血的胎儿。
1 常见的地中海贫血类型与临床症状
目前临床上常见的中间型地中海贫血有三大类：β-地中海贫血杂合子、HbE/β-双重杂合子地中海贫血和α-地中海贫血。中间型地中海贫血的贫血程度较重型轻，由于此类患者贫血，血红蛋白波动在60～100 g/L，机体携氧和抵抗力比正常人要偏差，同时机体反复慢性溶血，因此容易继发一系列急慢性病变。, 百拇医药(雷宇 曾清 程纬民)