hereditary persistence of fetal hemoglobin


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hereditary persistence of fetal hemoglobin

Hbα2γ2 An AD condition caused by a defect in the Hb 'switch' mechanism, where the usual transition from γ to β chain production does not occur; in HPFH, the number of cells with Hb F in the maternal circulation is greater than the baby's total blood volume, and therefore the condition's presence is suspected by mere calculations of maternal and fetal volumes; Type I–uniform distribution of HbF in all RBCs, as in Greek, Kenyan and Black American forms 2. Type II–heterogeneous distribution of HbF in RBCs, as in British and Swiss forms. See Class switching, Fetal hemoglobin.
References in periodicals archive ?
Zhao et al., "T to C substitution at -175 or -173 of the [gamma]-globin promoter affects GATA-1 and Oct1 binding in vitro differently but can independently reproduce the hereditary persistence of fetal hemoglobin phenotype in transgenic mice," The Journal of Biological Chemistry, vol.
Weatherall, "Hereditary persistence of fetal hemoglobin (HPFH) and Sfi thalassemia," British Journal of Haematology, vol.
Hereditary persistence of fetal hemoglobin presenting as fetal-maternal hemorrhage.

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