hemoglobin Lepore


Also found in: Dictionary, Thesaurus, Encyclopedia.

he·mo·glo·bin Le·pore

[MIM*142000-various]
a group of abnormal Hb's with normal α chains, but the non-α chains consist of the N-terminal portion of the δ chain joined to the C-terminal portion of the β chain, apparently as the result of nonhomologous pairing and crossing over between the genes for β and δ chains. The major types are Hb LeporeBoston (identical to Hb LeporeWashington), Hb LeporeHollandia, and Hb LeporeBaltimore, which differ in the region of crossing over (δ87-β116, δ22-β50, and δ50-β86, respectively). Heterozygotes form about 10% Hb Lepore, normal amounts of Hb A2, and moderately increased amounts of Hb F and usually have mild anemia, microcytosis, and hypochromia; homozygotes form only Hb Lepore and Hb F and have severe anemia. Compare: hemoglobin anti-Lepore.
[named for the family in which the variant was first discovered]

hemoglobin Lepore

A variant hemoglobin formed by an unequal crossover and fusion of the beta and delta genes. A single copy of the variant gene causes thalassemia minor. Homozygotes have thalassemia intermedia.
See also: hemoglobin