CTLA-4 Haploinsufficiency in a Patient with an Autoimmune Lymphoproliferative Disorder

Capsule Summary: - CTLA-4 haploinsufficiency appears to have characteristic histopathological findings that are distinct from ALPS. - Assessment of TFH, TFR and Tregs may serve as a biomarker profile in individuals with CTLA-4 mutations to predict disease.

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Bibliographic Details
Published inJournal of allergy and clinical immunology Vol. 140; no. 3; pp. 862 - 864.e4
Main Authors Kucuk, Zeynep Yesim, MD, Charbonnier, Louis-Marie, PhD, McMasters, Richard L., MD, Chatila, Talal, MD MSc, Bleesing, Jack J.H., MD PhD
Format Journal Article
LanguageEnglish
Published United States Elsevier Inc 01.09.2017
Elsevier Limited
Subjects
Allergy and Immunology
Antigens
Apoptosis
Biopsy
CTLA-4 protein
Defects
Haploinsufficiency
Histopathology
Lymphocytes
Lymphoma
Mutation
Patients
Primary immunodeficiencies
Studies
T cell receptors
ALPS
Impaired Treg suppression
Expanded germinal centers
CTLA-4 mutation
Autoimmune Lymphoproliferative Syndrome
Follicular helper T cells
Follicular regulatory T cells
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Summary:Capsule Summary: - CTLA-4 haploinsufficiency appears to have characteristic histopathological findings that are distinct from ALPS. - Assessment of TFH, TFR and Tregs may serve as a biomarker profile in individuals with CTLA-4 mutations to predict disease.
ISSN:0091-6749
1097-6825
DOI:10.1016/j.jaci.2017.02.032