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Hereditary Elliptocytosis: A Report of Two Families from New Guinea
Author(s) -
Pryor D. S.,
Pitney W. R.
Publication year - 1967
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1967.tb08701.x
Subject(s) - erythrocyte fragility , red cell , red blood cell , medicine , splenectomy , pediatrics , immunology , hemolysis , spleen
SUMMARY Two New Guinea families with hereditary elliptocytosis are reported. All four parents demonstrated elliptocytes in blood films and the two fathers also had deficiency of red‐cell glucose‐6‐phosphate dehydrogenase activity. The parents of one family showed normal red‐cell survival, while there was evidence of increased red‐cell destruction in the parents of the other family. One child in each family had severe haemolytic anaemia and it is suggested that these were possibly homozygous for elliptocytosis. Splenectomy was performed in one case and resulted in clinical and haematological improvement. Red‐cell osmotic fragility curves performed on members of both families showed some differences from the patterns previously reported in hereditary elliptocytosis.