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MM Udden, M Umeda, Y Hirano and DM Marcus
The In(Lu) phenotype is inherited as an autosomal dominant trait and is
characterized by suppression of the Lutheran, P1, i, and Aua erythrocyte
blood group antigens. We have developed a monoclonal antibody (L21) that
strongly agglutinates all erythrocytes except In(Lu), and we have
identified eight In(Lu) individuals among 42,000 blood donors tested.
Studies of two families confirmed the dominant mode of inheritance and
revealed several new features of this phenotype. The erythrocytes of all
five affected individuals from the two families exhibited diminished
hemagglutination by the lectin concanavalin A, although they reacted
normally with several other lectins. The erythrocytes of two affected
individuals in one family exhibited marked acanthocytosis. The erythrocytes
of the proposita of the other family exhibited a mild degree of
poikilocytosis, but the cells of the other two affected individuals in this
family had normal morphology. The osmotic fragility of fresh In(Lu)
erythrocytes was normal, but after incubation for 24 hours at 37 degrees C
in plasma the In(Lu) cells exhibited a marked increase in resistance to
osmotic lysis. During the incubation period the erythrocytes lost K+ and
their total cation content was diminished. These data indicate that in
addition to the suppression of blood group antigens noted previously, the
In(Lu) phenotype includes a variety of morphological abnormalities and a
defect in electrolyte metabolism. The use of L21 and similar monoclonal
antibodies provides a more sensitive means of detecting In(Lu) erythrocytes
than typing with human anti-Lub antisera.
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| Copyright © 1987 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||