Abstract

Hb Groene Hart [α119(H2)Pro→Ser, CCT→TCT (α1)] has been reported in heterozygotes of Moroccan origin and also in association with the common −α3.7 deletion. In all cases, the mutated protein was not detectable but was apparently associated with a mild α-thalassemia (thal) phenotype, presumably due to a modification of the α-globin chain domain that is recognized by the a hemoglobin stabilizing protein (AHSP). The present case of Hb Groene Hart homozygosity, confirms that the α-thal phenotype is associated with this α-globin chain. Hb Groene Hart must be quite frequent not only in Morocco but probably also among the northern African coastal population.

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