What are Hemoglobin H inclusion bodies?

Hemoglobin H bodies. Hemoglobin H, consisting of beta chain tetramers, is an unstable hemoglobin which forms precipitates just below the red blood cell membrane. This precipitated hemoglobin inclusion can be observed when red blood cells are stained with Brilliant Cresyl Blue (BCB).

What does Hemoglobin H trace mean?

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.

What causes formation of HbH inclusions?

HbH disease is caused by deletion of three α-globin genes. It is most common in Asian populations. With only one normal α-globin gene, the synthesis of α chains is markedly reduced, and tetramers of β-globin, called HbH, form.

How is Hemoglobin H diagnosed?

HbH disease should be considered in infants or children with mild-to-moderate microcytic hypochromic hemolytic anemia and hepatosplenomegaly. Heinz bodies can be detected on blood smears after cresyl blue staining. Hb biochemical analysis reveals the presence of HbH (5-30%). Diagnosis is confirmed by genetic testing.

What genotype represents HbH disease?

Population genetics Of those patients, 266 patients or 83% (95% confidence interval [CI], 79%-87%) have deletional Hb H disease. The most common genotype is (–SEA/-α3.7) found in 175 patients (55%), followed by (–SEA/-α4.2) 37 patients (12%), and (–FIL/ -α3.7) 36 patients (11%).

Is hemoglobin H disease serious?

Hemoglobin H disease. This type causes moderate to severe symptoms. These include lack of energy (fatigue) and exercise intolerance. You may also have an enlarged liver or spleen, yellowish skin, and leg ulcers. You have a greater risk of having a child with the most severe type, alpha thalassemia major.

What are the inclusion bodies in HbH disease composed of?

Heinz bodies (also referred to as “Heinz-Ehrlich bodies”) are inclusions within red blood cells composed of denatured hemoglobin. They are not visible with routine blood staining techniques, but can be seen with supravital staining.

What are the symptoms of Hemoglobin H disease?

What genotype represents Hb H disease?

How do you treat hemoglobin H?

Blood transfusions (as needed) Surgery to remove your spleen. Medicines to reduce extra iron from your body (called iron chelation therapy) Avoidance of certain oxidant drugs in hemoglobin H disease.