hemoglobin D disease

Dear doctor, Hemoglobin (Hb), as we know, is found in red blood cells, and helps in oxygen transport. The normal adult type is called Hb A. There are many other types of hemoglobins which may be found in humans, and which structurally differ from Hb A in very subtle ways. These variants of the normal hemoglobin constitute a group of diseases together called the hemoglobinopathies. These include sickle cell disease (Hb S), Hb C, Hb D, Hb E, etc. Hb D is a variant type of Hb, in which glutamine replaces glutamic acid at 121 position on beta chain. It is a rare disease, seen usually in people of Afghan, Pakistani, or North-West Indian (especially Sikhs from Punjab) descent. Among the subtypes of Hb D, the Hb Punjab / Hb Los Angeles is the commonest. There are four presentations of Hb D: Hb D trait, mixed Hb D - thalassemia, mixed Hb S - Hb D disease and the rare homozygous Hb D disease. Hb D may be asymptomatic; the homozygous forms may be associated with mild hemolytic anemia and mild to moderate splenomegaly. Reference: http://www.ispub.com/ostia/index.php?xmlFilePath=journals/ijpa/vol3n1/hbd.xml

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