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We describe a 2 year old girl with nucleoside phosphorylase (PNP) deficiency, who had low blood T cell numbers and T lymphocyte blastogenic response to mitogens, hypergammaglobulinaemia, high titres of antibodies to many common antigens, various autoantibodies, a monoclonal IgM-kappa protein, an increased frequency of mature Ig containing blood B cells and a high production of Ig in vitro in unstimulated cultures. E rosetting cells showed faint or no immunofluorescence staining with monoclonal antibodies directed against T cell membrane antigens. In vitro Ig production in response to pokeweed mitogen was defective, and no T cell helper or suppressor activity was observed. It is suggested that the immunoregulatory deficiency might have caused the B cell hyperactivity.
J M Zabay, and
E G De La Concha, and
C Ludeña, and
C Lozano, and
D Pascual-Salcedo, and
A Bootello, and
P Gonzalezporqué
December 1978,
The Journal of pediatrics,
J M Zabay, and
E G De La Concha, and
C Ludeña, and
C Lozano, and
D Pascual-Salcedo, and
A Bootello, and
P Gonzalezporqué
May 1975,
Lancet (London, England),
J M Zabay, and
E G De La Concha, and
C Ludeña, and
C Lozano, and
D Pascual-Salcedo, and
A Bootello, and
P Gonzalezporqué
March 1978,
The Journal of pediatrics,
J M Zabay, and
E G De La Concha, and
C Ludeña, and
C Lozano, and
D Pascual-Salcedo, and
A Bootello, and
P Gonzalezporqué
January 1977,
Advances in experimental medicine and biology,
J M Zabay, and
E G De La Concha, and
C Ludeña, and
C Lozano, and
D Pascual-Salcedo, and
A Bootello, and
P Gonzalezporqué
June 1979,
Journal of immunology (Baltimore, Md. : 1950),
J M Zabay, and
E G De La Concha, and
C Ludeña, and
C Lozano, and
D Pascual-Salcedo, and
A Bootello, and
P Gonzalezporqué
July 1978,
Clinical immunology and immunopathology,
J M Zabay, and
E G De La Concha, and
C Ludeña, and
C Lozano, and
D Pascual-Salcedo, and
A Bootello, and
P Gonzalezporqué
August 2012,
The Journal of allergy and clinical immunology,
J M Zabay, and
E G De La Concha, and
C Ludeña, and
C Lozano, and
D Pascual-Salcedo, and
A Bootello, and
P Gonzalezporqué
January 1977,
Advances in experimental medicine and biology,
J M Zabay, and
E G De La Concha, and
C Ludeña, and
C Lozano, and
D Pascual-Salcedo, and
A Bootello, and
P Gonzalezporqué
June 2002,
Pediatrics international : official journal of the Japan Pediatric Society,
J M Zabay, and
E G De La Concha, and
C Ludeña, and
C Lozano, and
D Pascual-Salcedo, and
A Bootello, and
P Gonzalezporqué
February 1977,
Clinica chimica acta; international journal of clinical chemistry,
