HbH stands for Hemoglobin H disease. It's a type of alpha-thalassemia, a blood disorder where there's reduced production of alpha-globin chains, a crucial component of hemoglobin, the protein responsible for carrying oxygen in red blood cells. This reduction leads to the formation of an abnormal hemoglobin called Hemoglobin H, hence the name HbH disease.
Understanding Hemoglobin H Disease (HbH)
HbH disease is characterized by:
- Chronic hemolytic anemia: The red blood cells break down prematurely, leading to anemia (low red blood cell count).
- Mild to severe anemia: The severity varies among individuals.
- Microcytosis and hypochromia: Red blood cells are smaller (microcytosis) and paler (hypochromia) than normal.
- Increased hemolysis: Increased destruction of red blood cells.
There are two main types of HbH disease:
- Deletional: Caused by genetic deletions.
- Non-deletional: Caused by non-deletion mutations.
HbH disease is considered a form of thalassemia intermedia, meaning it's a moderately severe form of thalassemia, falling between the milder forms and the more severe beta-thalassemia major. It requires careful management due to its chronic nature and potential complications. Treatment often involves managing the anemia and its symptoms.
The different sources highlight HbH disease as a form of alpha-thalassemia causing a chronic hemolytic anemia due to reduced alpha-globin chain formation. The unusual hemoglobin H is detected electrophoretically in peripheral blood. The severity can range from mild to severe.
