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Hereditary persistence of fetal hemoglobin

1960; Elsevier BV; Volume: 29; Issue: 1 Linguagem: Inglês

10.1016/0002-9343(60)90003-6

1555-7162

Emery C. Herman, C. Lockard Conley,

Iron Metabolism and Disorders

An abnormality of the red cells manifested by persistence of fetal hemoglobin was traced through three generations of a Negro family. This anomaly appears to be genetically determined by a factor allelic with the genes for hemoglobins A, S and C. The primary effect of this genetic disorder is unknown, but quite possibly is a failure of production of hemoglobin A, with formation of fetal hemoglobin occurring as an indirect result. In members of the family who showed persistence of fetal hemoglobin together with hemoglobin A, there were no clinical or hematologic abnormalities other than the presence of fetal hemoglobin in high concentration. Three members of the family had inherited the gene for hemoglobin S in addition to that resulting in persistence of fetal hemoglobin. These persons appeared to be well and had none of the manifestations of sickle cell anemia. Nevertheless their red cells contained predominantly hemoglobin S, and hemoglobin A could not be detected in their red cell hemolysates. The benign nature of this disorder is probably attributable to the presence of fetal hemoglobin in high concentration in the red cells and the resultant resistance to sickling.