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Haematological Changes Associated with the McLeod Phenotype of the Kell Blood Group System
Author(s) -
Wimer B. M.,
Marsh W. L.,
Taswell H. F.,
Galey W. R.
Publication year - 1977
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.1365-2141.1977.tb00642.x
Subject(s) - locus (genetics) , phenotype , allele , biology , antigen , genetics , antigenicity , gene , red blood cell , population , red cell , microbiology and biotechnology , immunology , medicine , environmental health
The McLeod phenotype is inherited as an X‐linked characteristic. The red cells have weak antigenicity in the Kell blood group and lack Kx, a precursorlike substance that appears to be necessary for proper biosynthesis of Kell antigens. Kx antigen is also required for establishment of normal cell morphology. Absence of Kx antigen causes a membrane abnormality, in which the most prominent feature is acanthocytosis, and a compensated haemolytic state. The X‐linked gene that determines normal Kx production is called X 1 k . Inheritance of a variant allele at the Xk locus is responsible for lack of Kx synthesis and the McLeod phenotype. The Xk locus is inactivated by the Lyon effect, and female carriers of the variant gene exhibit blood group mosaicism in the Kell system and have a dual red cell population of acanthocytes and discocytes.