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MHC class II-negative severe combined immunodeficiency [1 record]

Record 1 1993-02-11

English

Subject field(s)
  • Immunology
DEF

An inherited severe combined immunodeficiency (SCID) in man ... characterized by an absence of membrane expression of HLA (human leucocyte antigen) class II molecules and a decreased expression of HLA class I molecules on peripheral blood leucocytes. These patients have a profound defect of antibody production and off cell-mediated immunity leading to infections which are often lethal. Synthesis of all HLA class II molecules was found absent and synthesis of HLA class I molecules decreased in patient leucocytes and PHA-induced blasts.

OBS

According to World Health Organization's nomenclature.

French

Domaine(s)
  • Immunologie
OBS

Déficit immunitaire caractérisé par la présence en nombre normal de toutes les populations lymphocytaires et macrophagiques impliquées dans les réponses immunitaires, mais celles-ci sont devenues incapables d'exercer leurs fonctions du fait d'un défaut d'expression membranaire des produits du complexe majeur d'histocompatibilité (antigènes HLA de classe I et de classe II).

Spanish

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