Original Research

Published on 29 Mar 2019

Mycobacteria-Specific T Cells May Be Expanded From Healthy Donors and Are Near Absent in Primary Immunodeficiency Disorders

in Primary Immunodeficiencies

Shabnum Patel
Haili Lang
Gelina Sani
Alexandra F. Freeman
Jennifer Leiding
Patrick J. Hanley
Conrad Russell Cruz
Melanie Grant
Yunfei Wang
Benjamin Oshrine

Frontiers in Immunology

doi 10.3389/fimmu.2019.00621

5,668 views
4 citations

Original Research

Published on 26 Mar 2019

Primary and Secondary Immunodeficiency Diseases in Oncohaematology: Warning Signs, Diagnosis, and Management

in Primary Immunodeficiencies

Silvia Sánchez-Ramón
Arancha Bermúdez
Luis Ignacio González-Granado
Carlos Rodríguez-Gallego
Ana Sastre
Pere Soler-Palacín
the ID-Signal Onco-Haematology Group
Luis Allende
Laia Alsina
Ana María Bielsa

Frontiers in Immunology

doi 10.3389/fimmu.2019.00586

39,379 views
49 citations

Original Research

Published on 21 Mar 2019

Natural Killer Cell Degranulation Defect: A Cause for Impaired NK-Cell Cytotoxicity and Hyperinflammation in Fanconi Anemia Patients

in Primary Immunodeficiencies

Snehal Shabrish
Madhura Kelkar
Niranjan Chavan
Mukesh Desai
Umair Bargir
Maya Gupta
Priti Mehta
Akanksha Chichra
Chandrakala S
Prasad Taur

Frontiers in Immunology

doi 10.3389/fimmu.2019.00490

5,532 views
5 citations

Original Research

Published on 19 Mar 2019

Clinical and Immunological Phenotype of Patients With Primary Immunodeficiency Due to Damaging Mutations in NFKB2

in Primary Immunodeficiencies

Christian Klemann
Nadezhda Camacho-Ordonez
Linlin Yang
Zoya Eskandarian
Jessica L. Rojas-Restrepo
Natalie Frede
Alla Bulashevska
Maximilian Heeg
Moudjahed Saleh Al-Ddafari
Julian Premm

Frontiers in Immunology

doi 10.3389/fimmu.2019.00297

18,868 views
111 citations

Original Research

Published on 18 Mar 2019

Mapping of Signaling Pathways Linked to sIgAD Reveals Impaired IL-21 Driven STAT3 B-Cell Activation

in Primary Immunodeficiencies

Andri L. Lemarquis
Fannar Theodors
Helga K. Einarsdottir
Bjorn R. Ludviksson

Frontiers in Immunology

doi 10.3389/fimmu.2019.00403

3,608 views
3 citations

Original Research

Published on 15 Mar 2019

Thymic Epithelium Abnormalities in DiGeorge and Down Syndrome Patients Contribute to Dysregulation in T Cell Development

in Primary Immunodeficiencies

Genni Enza Marcovecchio
Ileana Bortolomai
Francesca Ferrua
Elena Fontana
Luisa Imberti
Erika Conforti
Donato Amodio
Sonia Bergante
Giulia Macchiarulo
Veronica D'Oria

Frontiers in Immunology

doi 10.3389/fimmu.2019.00447

11,825 views
70 citations

Original Research

Published on 14 Mar 2019

Cernunnos/Xlf Deficiency Results in Suboptimal V(D)J Recombination and Impaired Lymphoid Development in Mice

in Primary Immunodeficiencies

Benoit Roch
Vincent Abramowski
Julie Chaumeil
Jean-Pierre de Villartay

Frontiers in Immunology

doi 10.3389/fimmu.2019.00443

4,393 views
18 citations

Original Research

Published on 13 Mar 2019

Early Enzyme Replacement Therapy Improves Hearing and Immune Defects in Adenosine Deaminase Deficient-Mice

in Primary Immunodeficiencies

Xiaobai Xu
Jaina Negandhi
Weixian Min
Michael Tsui
Martin Post
Robert V. Harrison
Eyal Grunebaum

Frontiers in Immunology

doi 10.3389/fimmu.2019.00416

4,776 views
12 citations

Correction

Published on 11 Mar 2019

Corrigendum: Unperturbed Cytotoxic Lymphocyte Phenotype and Function in Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Patients

in Primary Immunodeficiencies

Jakob Theorell
Indre Bileviciute-Ljungar
Bianca Tesi
Heinrich Schlums
Mette Sophie Johnsgaard
Babak Asadi-Azarbaijani
Elin Bolle Strand
Yenan T. Bryceson

Frontiers in Immunology

doi 10.3389/fimmu.2019.00350

1,531 views
1 citation

Original Research

Published on 07 Mar 2019

Pathogenic Gene Mutations or Variants Identified by Targeted Gene Sequencing in Adults With Hemophagocytic Lymphohistiocytosis

in Primary Immunodeficiencies

Yi Miao
Hua-Yuan Zhu
Chun Qiao
Yi Xia
Yiling Kong
Yi-Xin Zou
Yu-Qing Miao
Xiao Chen
Lei Cao
Wei Wu

Frontiers in Immunology

doi 10.3389/fimmu.2019.00395

9,044 views
51 citations

Case Report

Published on 07 Mar 2019

Cytomegalovirus Colitis in Primary Hypogammaglobulinemia With Normal CD4+ T Cells: Deficiency of CMV-Specific CD8+ T Cells

in Primary Immunodeficiencies

Sudhanshu Agrawal
Amrita Khokhar
Sudhir Gupta

Frontiers in Immunology

doi 10.3389/fimmu.2019.00399

3,258 views
7 citations

Original Research

Published on 04 Mar 2019

The EuroFlow PID Orientation Tube for Flow Cytometric Diagnostic Screening of Primary Immunodeficiencies of the Lymphoid System

in Primary Immunodeficiencies

Mirjam van der Burg
Tomas Kalina
Martin Perez-Andres
Marcela Vlkova
Eduardo Lopez-Granados
Elena Blanco
Carolien Bonroy
Ana E. Sousa
Anne-Kathrin Kienzler
Marjolein Wentink

Frontiers in Immunology

doi 10.3389/fimmu.2019.00246

25,886 views
70 citations

Original Research

Published on 28 Feb 2019

Ultra-Sensitive CSF3R Deep Sequencing in Patients With Severe Congenital Neutropenia

in Primary Immunodeficiencies

Maksim Klimiankou
Murat Uenalan
Siarhei Kandabarau
Rainer Nustede
Ingeborg Steiert
Sabine Mellor-Heineke
Cornelia Zeidler
Julia Skokowa
Karl Welte

Frontiers in Immunology

doi 10.3389/fimmu.2019.00116

6,877 views
15 citations

Case Report

Published on 14 Feb 2019

Disseminated and Congenital Toxoplasmosis in a Mother and Child With Activated PI3-Kinase δ Syndrome Type 2 (APDS2): Case Report and a Literature Review of Toxoplasma Infections in Primary Immunodeficiencies

in Primary Immunodeficiencies

Djuro Karanovic
Ian C. Michelow
Anthony R. Hayward
Suk See DeRavin
Ottavia M. Delmonte
Michael E. Grigg
Adam Kerry Dobbs
Julie E. Niemela
Jennifer Stoddard
Zaid Alhinai

Frontiers in Immunology

doi 10.3389/fimmu.2019.00077

6,157 views
16 citations

Case Report

Published on 14 Feb 2019

First Case of Patient With Two Homozygous Mutations in MYD88 and CARD9 Genes Presenting With Pyogenic Bacterial Infections, Elevated IgE, and Persistent EBV Viremia

in Primary Immunodeficiencies

Maria Chiriaco
Gigliola Di Matteo
Francesca Conti
Davide Petricone
Maia De Luca
Silvia Di Cesare
Cristina Cifaldi
Rita De Vito
Matteo Zoccolillo
Jessica Serafinelli

Frontiers in Immunology

doi 10.3389/fimmu.2019.00130

4,438 views
17 citations

Review

Published on 12 Feb 2019

Primary Immunodeficiency and Cancer Predisposition Revisited: Embedding Two Closely Related Concepts Into an Integrative Conceptual Framework

in Primary Immunodeficiencies

Oskar A. Haas

Frontiers in Immunology

doi 10.3389/fimmu.2018.03136

16,222 views
52 citations

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Articles

Mycobacteria-Specific T Cells May Be Expanded From Healthy Donors and Are Near Absent in Primary Immunodeficiency Disorders

Primary and Secondary Immunodeficiency Diseases in Oncohaematology: Warning Signs, Diagnosis, and Management

Natural Killer Cell Degranulation Defect: A Cause for Impaired NK-Cell Cytotoxicity and Hyperinflammation in Fanconi Anemia Patients

Clinical and Immunological Phenotype of Patients With Primary Immunodeficiency Due to Damaging Mutations in NFKB2

Mapping of Signaling Pathways Linked to sIgAD Reveals Impaired IL-21 Driven STAT3 B-Cell Activation

Thymic Epithelium Abnormalities in DiGeorge and Down Syndrome Patients Contribute to Dysregulation in T Cell Development

Cernunnos/Xlf Deficiency Results in Suboptimal V(D)J Recombination and Impaired Lymphoid Development in Mice

Early Enzyme Replacement Therapy Improves Hearing and Immune Defects in Adenosine Deaminase Deficient-Mice

Corrigendum: Unperturbed Cytotoxic Lymphocyte Phenotype and Function in Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Patients

Pathogenic Gene Mutations or Variants Identified by Targeted Gene Sequencing in Adults With Hemophagocytic Lymphohistiocytosis

Cytomegalovirus Colitis in Primary Hypogammaglobulinemia With Normal CD4+ T Cells: Deficiency of CMV-Specific CD8+ T Cells

The EuroFlow PID Orientation Tube for Flow Cytometric Diagnostic Screening of Primary Immunodeficiencies of the Lymphoid System

Ultra-Sensitive CSF3R Deep Sequencing in Patients With Severe Congenital Neutropenia

Disseminated and Congenital Toxoplasmosis in a Mother and Child With Activated PI3-Kinase δ Syndrome Type 2 (APDS2): Case Report and a Literature Review of Toxoplasma Infections in Primary Immunodeficiencies

First Case of Patient With Two Homozygous Mutations in MYD88 and CARD9 Genes Presenting With Pyogenic Bacterial Infections, Elevated IgE, and Persistent EBV Viremia

Primary Immunodeficiency and Cancer Predisposition Revisited: Embedding Two Closely Related Concepts Into an Integrative Conceptual Framework