Primary Immune Regulatory Disorders With an Autoimmune Lymphoproliferative Syndrome-Like Phenotype: Immunologic Evaluation, Early Diagnosis and Management

Primary Immune Regulatory Disorders With an Autoimmune Lymphoproliferative Syndrome-Like Phenotype: Immunologic Evaluation, Early Diagnosis and Management

Primary immune regulatory disorders (PIRD) are associated with autoimmunity, autoinflammation and/or dysregulation of lymphocyte homeostasis. Autoimmune lymphoproliferative syndrome (ALPS) is a PIRD due to an apoptotic defect in Fas-FasL pathway and characterized by benign and chronic lymphoprolifer...

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Bibliographic Details
Main Authors: Marta López-Nevado, Luis I. González-Granado, Raquel Ruiz-García, Daniel Pleguezuelo, Oscar Cabrera-Marante, Nerea Salmón, Pilar Blanco-Lobo, Nerea Domínguez-Pinilla, Rebeca Rodríguez-Pena, Elena Sebastián, Jaime Cruz-Rojo, Peter Olbrich, Jesús Ruiz-Contreras, Estela Paz-Artal, Olaf Neth, Luis M. Allende
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-08-01
Series:Frontiers in Immunology
Subjects:
ALPS
ALPS-like
autoimmunity
lymphoproliferation
malignancy
immune dysregulation
Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2021.671755/full

Internet

https://www.frontiersin.org/articles/10.3389/fimmu.2021.671755/full

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