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. [1]

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. Pregnancy exacerbates the anaemia due to a dilutional effect.

Haemoglobin electrophoresis demonstrates presence of fast-moving HbH 5-15% of the total haemoglobin. The peaks of HbH and Hb Bart’s are also identified by automatic high-performance liquid chromatography (HPLC) and capillary electrophoresis system, however, the percentage may not be accurately quantitated.

2019年6月26日 · Located in Humber Bay Park East, the HBBH is a restored outdoor area which has been designed to attract butterflies by offering nectar plants, host plants, boulders for sunning, wind shelters, water access, hibernacula (places for hibernating) and other things butterflies need to survive through every stage of life.

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. Normally, there are four genes to produce alpha globin chains.

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 a serious health problem in Southeast Asia and southern China. Thousands of affected patients live in the Middle East, the Mediterranean region, and North America. Many patients require intermittent transfusions. The clinical severity is strongly influenced by the type of mutation.

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.

Hemoglobin H disease is a subtype of alpha-thalassemia (see 604131) in which patients have compound heterozygosity for alpha (+)-thalassemia, caused by deletion of one alpha-globin gene, and for alpha (0)-thalassemia, caused by deletion in cis of …

Clinical phenotypes are diverse among affected individuals with non-deletional haemoglobin H (HbH) disease (--/α T α). This mainly depends on the type of mutation in α-globin genes, whether the mutation is in the HBA1 or the HBA2 gene, as well as co-inheritance of β-thalassaemia.