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Spanish Rh null family caused by a silent Rh gene: Hematological, serological, and biochemical studies
Author(s) -
PérezPérez Carmen,
Taliano Victor,
Mouro Isabelle,
Huet Martine,
SalatMarti Antonio,
Martinez Antonio,
Rouger Philippe,
Cartron JeanPierre
Publication year - 1992
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830400412
Subject(s) - serology , antibody , glycoprotein , microbiology and biotechnology , rh blood group system , western blot , monoclonal antibody , hemolytic anemia , biology , gene , virology , genetics , immunology
Another example of rare red cells that failed to react with all anti‐Rh and anti‐LW antibodies was discovered in a Spanish woman suffering from a severe hemolytic anemia typical of the Rh null syndrome. Family study and Rh blood typings demonstrated clearly that the proposita was homozygous for a silent Rh gene complex (Rh null of the amorph type) that she inherited from her parents who are first cousins. Western blot analysis carried out with glycosylation‐independent antibodies directed against the Rh polypeptide and the LW glycoprotein, respectively, confirmed that these protein components were absent from the red cells of the proposita. In addition, the patient was typed U‐positive, again in agreement with the presence on her red cells of 45–75 kDa glycoproteins detected with the murine monoclonal antibody 2D10. © 1992 Wiley‐Liss, Inc.