HbH disease is characterised by a moderate anaemia (Hb typically ~80g/L, but can range from 60-100g/L). Haemolytic episodes are triggered by infections, especially parvovirus B19 infection and exposure to oxidant drugs.

患有轻中度小细胞低色素性溶血性贫血和肝脾肿大的婴儿或儿童应考虑 HbH 病。 甲酚蓝染色后可在血涂片上检测到亨氏小体。 Hb 生化分析显示存在 HbH (5-30%)。

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.

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2003年2月1日 · Hb H disease is commonly caused by a deletion removing both α-globin genes on one chromosome 16, plus a deletion removing only a single α-globin gene on the other chromosome 16 such as the (-α 3.7) or (-α 4.2) deletions. These are known as “deletional Hb H disease.” 11 15-33.

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.

HbH患者出生时与正常婴儿一样，未满1岁前多无贫血症状，以后随着年龄增长逐渐出现典型的HbH病特征，主要表现为轻至中度的慢性贫血。 约2/3以上患者有肝脾大，间歇发作轻度黄疸，但无地中海贫血外貌、骨骼系统变化轻微，生长发育正常，可长期存活。

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。

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.

正常人有4个α基因，有3个α基因存在缺陷时形成HbH病，可在患者外周血血红蛋白电泳中检测出血红蛋白H带，部分可见Hb Bart’s区带 。HbH病是能生存下来的α地贫个体中最严重的一种 ，基因型主要分为缺失型( --/-α)及非缺失型( --/α T α)。HbH病基因变异类型具有 ...