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 · In this chapter we will review the molecular defects leading to Hb H disease, and its clinical manifestations, diagnosis, and management. We will also discuss possible modifiers at both the genetic and environmental levels that may modify its clinical severity.

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.

The loss of function of three alpha-globin genes is called hemoglobin H disease. People with hemoglobin H disease have a variable phenotype that can range from mild symptoms to those similar to thalassemia major.

2003年2月1日 · Hb H disease is a form of α-thalassemia often manifested clinically as thalassemia intermedia with moderate anemia. It is commonly found in Southeast Asian, Middle Eastern, and Mediterranean populations. There is a wide spectrum of …

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.