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Hemoglobin H disease, also called alpha-thalassemia intermedia, is a disease affecting hemoglobin, the oxygen carrying molecule within red blood cells. It is a form of Alpha-thalassemia which most commonly occurs due to deletion of 3 out of 4 of the α-globin genes.

2009年1月1日 · Hemoglobin H (Hb H) disease is the most severe non-fatal form of α-thalassemia syndrome, mostly caused by molecular defects of the α-globin genes in which α-globin expression is decreased.

α-地中海贫血 (α-地贫，α-thalassemia) (OMIM #604131)是由于α珠蛋白肽链的合成受到部分或完全抑制，导致血红蛋白 (hemoglobin，Hb)合成不足而引发的遗传性溶血性贫血，呈常染色体隐性遗传，致病基因为HBA1 (OMIM *141800)和 HBA2 (OMIM *141850)，男女患病几率相等 [1, 2]。 α-地贫的临床表现变异度较大，病情的严重程度与α-珠蛋白链减少的程度直接相关 [3, 4, 5]。 携带者一般表型正常，无症状或仅有轻微的红细胞参数改变。 患者按症状轻重可分为Hb H病和Hb …

2023年9月4日 · Hemoglobin H disease (alpha-thalassemia) can be found in patients that live in areas with a high incidence of malaria. Symptoms at presentation, such as episodic anemia, and appropriate diagnostic testing, such as hematology studies and electrophoresis, are essential to timely and proper treatment.

血红蛋白 H 病 (HbH) 病属于非输血依赖性地中海贫血组。 HbH 疾病通常由三个 α-珠蛋白等位基因失活导致 Hb 的 α-珠蛋白链产生不足，并形成 β-4 四聚体 (HbH)。 HbH四聚体对氧具有高亲和力，并且高度不稳定，沉淀为在成熟红细胞中占主导地位的有毒亨氏体，导致过早溶血而不是无效红细胞生成。 它在氧化应激下增加，这解释了与感染或摄入氧化剂药物相关的高溶血。 该疾病通常是由任一 α 球蛋白基因（HBA1和HBA2 ）中的复合杂合或纯合变异引起的; 16p13.3），伴随 …

Hemoglobin H disease (HbH) is a form of alpha thalassemia in which moderately severe anemia develops due to reduced formation of alpha globin chains. In this condition, as in the other forms of thalassemia, there is an imbalance of globin chains needed to form hemoglobin.

Hemoglobin H disease is named for the abnormal hemoglobin H (created by the remaining beta globin) that destroys red blood cells. Alpha-thalassemia is an inherited blood disorder that reduces the body’s production of hemoglobin. Affected people have anemia, which can cause pale skin, weakness, fatigue, and more serious complications.

2009年1月26日 · Hemoglobin H (Hb H) disease is the most common form of thalassemia intermedia and has many features that require careful consideration in management. In the majority of cases, Hb H disease results from double heterozygosity for alpha(0)-thalassemia due to deletions that remove both linked alpha-glob …

HbH病可分为缺失型与非缺失型,在我国最常见非缺失型是Hb CS (Hemoglobin Constant Spring),非缺失型患者的症状往往更重。 可以通过检测血红蛋白 (Hb)、红细胞计数 (RBC)、红细胞的平均体积 (MCV)、平均血红蛋白浓度 (MCH)及红细胞分布宽度 (RDW)对HbH 病患者进行筛查,通过基因分析可以确诊, 早期的诊断,对于疾病的管理和预后有着重要的意义。 HbH 病的治疗以预防和支持治疗为主,必要时进行输血、铁螯合治疗及脾切除治疗,实验证明中医治疗对HbH 病有一定效果。