Combined T Cell and B Cell Deficiency – SCID Forms: T−B+

Détails

ID Serval
serval:BIB_C922879F01CD
Type
Partie de livre
Collection
Publications
Titre
Combined T Cell and B Cell Deficiency – SCID Forms: T−B+
Titre du livre
Encyclopedia of Immunobiology
Auteur(s)
Candotti Fabio
Editeur
Academic Press
Lieu d'édition
Oxford
ISBN
978-0-08-092152-5
Statut éditorial
Publié
Date de publication
2016
Peer-reviewed
Oui
Pages
360-368
Langue
anglais
Résumé
Abstract Severe combined immunodeficiencies (SCIDs) are a heterogeneous group of diseases characterized by profound compromise of both T lymphocyte and B lymphocyte numbers and/or function. From a descriptive standpoint, SCID can be categorized based on the composition of the peripheral blood lymphocyte populations observed in affected patients. While many forms of SCID present with both T cell and B cell lymphopenia (T−B− SCID), in one group of such disorders B cell differentiation is conserved and normal or even elevated numbers of peripheral blood B lymphocytes can be observed. These diseases are called SCID T−B+ and include the X-linked form of SCID (SCIDX-1), the deficiency of Janus kinase family member 3 (JAK3-SCID), and the defect of expression of the alpha chain of the interleukin-7 receptor (IL7-Rα-SCID). This article describes the main features of these rare clinical entities.
Mots-clé
Cytokine, Gene, Gene therapy, Immunity, Immunodeficiency, Infection, Lymphocyte, Mutation, Receptor, Signaling, Transplantation, X-chromosome
Création de la notice
07/11/2017 19:42
Dernière modification de la notice
11/09/2019 6:26
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