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Hereditary persistence of fetal hemo globin found frequently among the japanese

Kaneto, Y.

Shikoku Acta Medica 37(2): 334-344

1981

Accession: 005563441

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Summary
The number of subjects with hereditary persistence of fetal hemoglobin (HPFH) (Tokushima variant) reported previously was extended and results of further investigations were described in detail. The study included 300 healthy adults (150 males and 150 females). HbF (fetal Hb)-containing erythrocytes (F-cells) in blood smears were detected by indirect immunofluorescent antibody technique using anti-human HbF rabbit .gamma.-globulins, and F-cell percentage was calculated as the percentage of fluorescent erythrocytes (or F-cells) among the total erythrocytes. Healthy adults were classified into 2 groups (low F-cell group and high F-cell group) by the F-cell percentage. F-cell percentage was 2.1 .+-. 0.9 (mean .+-. SD) in the low F-cell group and 8.4 .+-. 2.7 in the high F-cell group. The high F-cell group may be called a HPFH. The incidence among healthy adults was 11.3% in males, 20.7% in females and 16.0% in total. HbF content in hemolysate determined by the alkali denaturation method was elevated to 1.40 .+-. 0.63% in HPFH. Hbf detected by the immunofluorescent antibody technique was heterogeneously distributed among erythrocytes. Decrease of HbA2 was not detected. No clinical or hematological abnormalities were observed in the HPFH. The HPFH was supposedly inherited as an X-linked dominant trait. The incidence in each sex is consistent with the expected value of X-linked dominant inheritance. Parents of high F-cell individuals from 21 pedigrees were investigated with the result that, except for 1 pedigree, either of the parents had high F-cell value, that no transmission from father to son was seen and that all daughters of high F-cell males had high F-cell values. The recombination ratio between the genes of the HPFH and color blindness was 18%, suggesting the short distance between the 2 genes on X chromosome. The HPFH is similar to the Swiss type, but is distinct from the Negro or the Greek type. The incidence of the HPFH, 16%, was remarkably high compared with that of the Swiss type, 1%. X-linked dominant inheritance is another characteristic. The HPFH was discovered in Tokushima but will be seen all over Japan with almost the same incidence. It may also be found all over the world, if not with the same incidence.

References

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