Hereditary Elliptocytosis in Iceland
SUMMARY An Icelandic family with 50 elliptocytic individuals is surveyed. Pedigree studies indicate strongly that affected members of the family are descendants of a common ancestor. The hereditary pattern is typical for a dominant, autosomal gene with full penetrance. A high incidence (over 57 per...
| Published in: | British Journal of Haematology |
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| Main Authors: | , , |
| Format: | Article in Journal/Newspaper |
| Language: | English |
| Published: |
Wiley
1967
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| Subjects: | |
| Online Access: | http://dx.doi.org/10.1111/j.1365-2141.1967.tb08855.x https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1111%2Fj.1365-2141.1967.tb08855.x https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1365-2141.1967.tb08855.x |
| Summary: | SUMMARY An Icelandic family with 50 elliptocytic individuals is surveyed. Pedigree studies indicate strongly that affected members of the family are descendants of a common ancestor. The hereditary pattern is typical for a dominant, autosomal gene with full penetrance. A high incidence (over 57 per cent) of signs and/or symptoms of haemolysis is found among the affected at one time or another. No haptoglobin was detected in 80 per cent of affected individuals investigated. The age distribution of the affected at the time of diagnosis shows an increased incidence of anaemia with increasing age. In three affected individuals splenectomy has been performed with satisfactory results. The variation in the grades of haemolysis and the high incidence of haemolysis found in the affected family members are discussed. |
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