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hemoglobin H disease

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hemoglobin H disease
n.
An inherited disease characterized by moderate anemia and red blood cell abnormalities, with red blood cells containing Bart's hemoglobin being replaced by cells containing hemoglobin H.
Hemoglobin H disease
A thalassemia-like syndrome causing moderate anemia and red blood cell abnormalities.
Mentioned in: Hemoglobin Electrophoresis
hemoglobin H disease,
alpha-thalassemia in individuals heterozygous for hemoglobin H, characterized by chronic hemolytic anemia associated with splenomegaly. Red blood cell hypochromia, anisocytosis, and poikilocytosis are accompanied by inclusion bodies detectable by supravital staining.


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Deletion of three alpha globin genes results in Hemoglobin H disease, with 4-30% rapidly migrating hemoglobin H and a small amount of hemoglobin Bart's.
PAT-3. Hemoglobin H disease mimics a normal pattern on hemoglobin ... by Posey, Y.F. / Southern Medical Journal
 
 
 
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