What is the meaning of Hemoglobin H disease?

What is the meaning of Hemoglobin H disease?

Disease definition. An intermediate form of alpha-thalassemia characterized by increased hemolysis and mild to severe anemia with marked microcytosis and hypochromia.

What causes Hb H disease?

HbH disease is caused by a loss of three of the four alpha-globin alleles. In these two conditions, a shortage of alpha-globin prevents cells from making normal hemoglobin. Instead, cells produce abnormal forms of hemoglobin called hemoglobin Bart (Hb Bart) or hemoglobin H (HbH).

What is the defect usually found in Hb H disease?

In Hb H disease, three of four α-globin genes are defective, and only one α-globin gene is functional. There are two types of Hb H disease: deletional (genotype –/- α ) and nondeletional (genotypes –/αTα).

What kind of disease is Hb H disease?

In Hb H disease, three of four α-globin genes are defective, and only one α-globin gene is functional. There are two types of Hb H disease: deletional (genotype –/- α ) and nondeletional (genotypes –/αTα). The latter type can have a more severe phenotype than the former.

What happens to the body with HbH disease?

HbH disease causes mild to moderate anemia, hepatosplenomegaly, and yellowing of the eyes and skin (jaundice). Some affected individuals also have bone changes such as overgrowth of the upper jaw and an unusually prominent forehead.

What kind of anemia does HbH disease cause?

HbH disease may cause mild to moderate anemia; hepatosplenomegaly; jaundice; or bone changes. Alpha-thalassemia typically results from deletions involving the HBA1 and HBA2 genes. The inheritance is complex, and can be read about here.

How is haemoglobin H ( HbH ) disease characterised?

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.

What causes high hemoglobin levels?

Causes Of High Hemoglobin Levels In Blood. Common causes for a high level of haemoglobin in your blood include: Smoking. Living at higher altitudes; the manufacture of RBCs tends to increase in order to recompense for the low oxygen supply at that height.

When is high hemoglobin dangerous?

High hemoglobin levels could be indicative of the rare blood disease, polycythemia . It causes the body to make too many red blood cells, causing the blood to be thicker than usual. This can lead to clots, heart attacks, and strokes. It is a serious lifelong condition that can be fatal if it is not treated.

What are the side effects of high hemoglobin?

You may not experience any symptoms if your hemoglobin level is mildly elevated. But when it is too high, symptoms that occur can include headaches, vision problems, fatigue, dizziness, periods of mental confusion, and abdominal discomfort. You may also experience nosebleeds and flushing in your feet, hands, and face.

What medications cause low hemoglobin?

To avoid such cases from occurring, the causes of Low Hemoglobin levels should be known and treated or controlled if possible. Low Hemoglobin levels can be a result of using drugs such like Aspirin, Antibiotics, Antineoplastic drugs, Indomethacin, Rifampin, and Sulfonamides.