MaghrebDataLibMedV2, Main, Exploration, bibRecord, 000547

Outcomes and treatment strategies for autoimmunity and hyperinflammation in patients with RAG deficiency.

Identifieur interne : 000547 ( Main/Exploration ); précédent : 000546; suivant : 000548

Outcomes and treatment strategies for autoimmunity and hyperinflammation in patients with RAG deficiency.

Auteurs : Jocelyn R. Farmer ; Zsofia Foldvari ; Boglarka Ujhazi ; Suk See De Ravin ; Karin Chen ; Jack J. H. Bleesing ; Catharina Schuetz ; Waleed Al-Herz ; Roshini Sarah Abraham ; Avni Y. Joshi ; Beatriz T. Costa-Carvalho ; David Buchbinder ; Claire Booth ; Andreas Reiff ; Polly J. Ferguson ; Asghar Aghamohammadi ; Hassan Abolhassani ; Jennifer M. Puck ; Mehdi Adeli ; Caterina Cancrini ; Paolo Palma ; Alice Bertaina ; Franco Locatelli ; Gigliola Di Matteo ; Raif S. Geha ; Maria G. Kanariou ; Evangelia Lycopoulou ; Marianna Tzanoudaki ; John W. Sleasman ; Suhag Parikh ; Gloria Pinero ; Bernard M. Fischer ; Ghassan Dbaibo ; Ekrem Unal ; Turkan Patiroglu ; Musa Karakukcu ; Khulood Khalifa Al-Saad ; Meredith A. Dilley ; Sung-Yun Pai ; Cullen M. Dutmer ; Erwin W. Gelfand ; Christoph B. Geier ; Martha M. Eibl ; Hermann M. Wolf ; Lauren A. Henderson ; Melissa M. Hazen ; Carmem Bonfim ; Beata Wolska-Ku Nierz ; Manish J. Butte ; Joseph D. Hernandez ; Sarah K. Nicholas ; Polina Stepensky ; Shanmuganathan Chandrakasan ; Maurizio Miano ; Emma Westermann-Clark ; Vera Goda ; Gergely Kriván ; Steven M. Holland ; Olajumoke Fadugba ; Sarah E. Henrickson ; Ahmet Ozen ; Elif Karakoc-Aydiner ; Safa Baris ; Ayca Kiykim ; Robbert Bredius ; Birgit Hoeger ; Kaan Boztug ; Olga Pashchenko ; Benedicte Neven ; Despina Moshous ; Jean-Pierre De Villartay ; Ahmed Aziz Bousfiha ; Harry R. Hill ; Luigi D. Notarangelo ; Jolan E. Walter

Source :

The journal of allergy and clinical immunology. In practice [ 2213-2198 ] ; 2019.

RBID : PMC:6612449

Descripteurs français

KwdFr :
- Adolescent (MeSH), Adulte (MeSH), Adulte d'âge moyen (MeSH), Auto-immunité (MeSH), Déficits immunitaires (immunologie), Déficits immunitaires (thérapie), Enfant (MeSH), Enfant d'âge préscolaire (MeSH), Femelle (MeSH), Humains (MeSH), Immunosuppresseurs (usage thérapeutique), Inflammation (MeSH), Jeune adulte (MeSH), Mâle (MeSH), Nourrisson (MeSH), Protéines à homéodomaine (MeSH), Résultat thérapeutique (MeSH), Transplantation de cellules souches hématopoïétiques (MeSH).
MESH :
- immunologie : Déficits immunitaires.
- thérapie : Déficits immunitaires.
- usage thérapeutique : Immunosuppresseurs.
- Adolescent, Adulte, Adulte d'âge moyen, Auto-immunité, Enfant, Enfant d'âge préscolaire, Femelle, Humains, Inflammation, Jeune adulte, Mâle, Nourrisson, Protéines à homéodomaine, Résultat thérapeutique, Transplantation de cellules souches hématopoïétiques.

English descriptors

KwdEn :
- Adolescent (MeSH), Adult (MeSH), Autoimmunity (MeSH), Child (MeSH), Child, Preschool (MeSH), Female (MeSH), Hematopoietic Stem Cell Transplantation (MeSH), Homeodomain Proteins (MeSH), Humans (MeSH), Immunologic Deficiency Syndromes (immunology), Immunologic Deficiency Syndromes (therapy), Immunosuppressive Agents (therapeutic use), Infant (MeSH), Inflammation (MeSH), Male (MeSH), Middle Aged (MeSH), Treatment Outcome (MeSH), Young Adult (MeSH).
MESH :
- chemical , therapeutic use : Immunosuppressive Agents.
- chemical : Homeodomain Proteins.
- immunology : Immunologic Deficiency Syndromes.
- therapy : Immunologic Deficiency Syndromes.
- Adolescent, Adult, Autoimmunity, Child, Child, Preschool, Female, Hematopoietic Stem Cell Transplantation, Humans, Infant, Inflammation, Male, Middle Aged, Treatment Outcome, Young Adult.

Abstract

Background:

While autoimmunity and hyperinflammation secondary to recombinase activating gene (RAG) deficiency have been associated with delayed diagnosis and even death, our current understanding is limited primarily to small case series.

Objective:

Understand the frequency, severity, and treatment responsiveness of autoimmunity and hyperinflammation in RAG deficiency.

Methods:

In reviewing the literature and our own database, we identified 85 patients with RAG deficiency, reported between 2001 and 2016, and compiled the largest case series to date of 63 patients with prominent autoimmune and/or hyperinflammatory pathology.

Results:

Diagnosis of RAG deficiency was delayed a median of 5 years from the first clinical signs of immune dysregulation. The majority of patients (55.6%) presented with more than one autoimmune or hyperinflammatory complication, with the most common etiologies being cytopenias (84.1%), granulomas (23.8%), and inflammatory skin disorders (19.0%). Infections, including live viral vaccinations, closely preceded the onset of autoimmunity in 28.6% of cases. Autoimmune cytopenias had early onset (median 1.9, 2.1, and 2.6 years for autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP) and autoimmune neutropenia (AN), respectively) and were refractory to intravenous immunoglobulin, steroids, and rituximab in the majority of cases (64.7%, 73.7%, and 71.4% for AIHA, ITP, and AN, respectively). Evans syndrome specifically was associated with lack of response to first-line therapy. Treatment-refractory autoimmunity/hyperinflammation prompted hematopoietic stem cell transplantation in 20 patients.

Conclusions:

Autoimmunity/hyperinflammation can be a presenting sign of RAG deficiency and should prompt further evaluation. Multi-lineage cytopenias are often refractory to immunosuppressive treatment and may require hematopoietic cell transplantation for definitive management.

Url:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6612449

DOI: 10.1016/j.jaip.2019.02.038
PubMed: 30877075
PubMed Central: 6612449

Affiliations:

Le document en format XML

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<author><name sortKey="Puck, Jennifer M" sort="Puck, Jennifer M" uniqKey="Puck J" first="Jennifer M." last="Puck">Jennifer M. Puck</name>
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<author><name sortKey="Adeli, Mehdi" sort="Adeli, Mehdi" uniqKey="Adeli M" first="Mehdi" last="Adeli">Mehdi Adeli</name>
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</affiliation>
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<author><name sortKey="Cancrini, Caterina" sort="Cancrini, Caterina" uniqKey="Cancrini C" first="Caterina" last="Cancrini">Caterina Cancrini</name>
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<affiliation><nlm:aff>NONE</nlm:aff>
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<author><name sortKey="Palma, Paolo" sort="Palma, Paolo" uniqKey="Palma P" first="Paolo" last="Palma">Paolo Palma</name>
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<author><name sortKey="Bertaina, Alice" sort="Bertaina, Alice" uniqKey="Bertaina A" first="Alice" last="Bertaina">Alice Bertaina</name>
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<author><name sortKey="Locatelli, Franco" sort="Locatelli, Franco" uniqKey="Locatelli F" first="Franco" last="Locatelli">Franco Locatelli</name>
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<affiliation><nlm:aff>NONE</nlm:aff>
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<author><name sortKey="Di Matteo, Gigliola" sort="Di Matteo, Gigliola" uniqKey="Di Matteo G" first="Gigliola" last="Di Matteo">Gigliola Di Matteo</name>
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<author><name sortKey="Geha, Raif S" sort="Geha, Raif S" uniqKey="Geha R" first="Raif S." last="Geha">Raif S. Geha</name>
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<author><name sortKey="Lycopoulou, Evangelia" sort="Lycopoulou, Evangelia" uniqKey="Lycopoulou E" first="Evangelia" last="Lycopoulou">Evangelia Lycopoulou</name>
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<author><name sortKey="Sleasman, John W" sort="Sleasman, John W" uniqKey="Sleasman J" first="John W." last="Sleasman">John W. Sleasman</name>
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<author><name sortKey="Parikh, Suhag" sort="Parikh, Suhag" uniqKey="Parikh S" first="Suhag" last="Parikh">Suhag Parikh</name>
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<author><name sortKey="Pinero, Gloria" sort="Pinero, Gloria" uniqKey="Pinero G" first="Gloria" last="Pinero">Gloria Pinero</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
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<author><name sortKey="Fischer, Bernard M" sort="Fischer, Bernard M" uniqKey="Fischer B" first="Bernard M." last="Fischer">Bernard M. Fischer</name>
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<author><name sortKey="Dbaibo, Ghassan" sort="Dbaibo, Ghassan" uniqKey="Dbaibo G" first="Ghassan" last="Dbaibo">Ghassan Dbaibo</name>
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<author><name sortKey="Patiroglu, Turkan" sort="Patiroglu, Turkan" uniqKey="Patiroglu T" first="Turkan" last="Patiroglu">Turkan Patiroglu</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
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<author><name sortKey="Karakukcu, Musa" sort="Karakukcu, Musa" uniqKey="Karakukcu M" first="Musa" last="Karakukcu">Musa Karakukcu</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Al Saad, Khulood Khalifa" sort="Al Saad, Khulood Khalifa" uniqKey="Al Saad K" first="Khulood Khalifa" last="Al-Saad">Khulood Khalifa Al-Saad</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Dilley, Meredith A" sort="Dilley, Meredith A" uniqKey="Dilley M" first="Meredith A." last="Dilley">Meredith A. Dilley</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
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<author><name sortKey="Pai, Sung Yun" sort="Pai, Sung Yun" uniqKey="Pai S" first="Sung-Yun" last="Pai">Sung-Yun Pai</name>
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</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
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<author><name sortKey="Dutmer, Cullen M" sort="Dutmer, Cullen M" uniqKey="Dutmer C" first="Cullen M." last="Dutmer">Cullen M. Dutmer</name>
<affiliation><nlm:aff>NONE</nlm:aff>
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<author><name sortKey="Gelfand, Erwin W" sort="Gelfand, Erwin W" uniqKey="Gelfand E" first="Erwin W." last="Gelfand">Erwin W. Gelfand</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Geier, Christoph B" sort="Geier, Christoph B" uniqKey="Geier C" first="Christoph B." last="Geier">Christoph B. Geier</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Eibl, Martha M" sort="Eibl, Martha M" uniqKey="Eibl M" first="Martha M." last="Eibl">Martha M. Eibl</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Wolf, Hermann M" sort="Wolf, Hermann M" uniqKey="Wolf H" first="Hermann M." last="Wolf">Hermann M. Wolf</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Henderson, Lauren A" sort="Henderson, Lauren A" uniqKey="Henderson L" first="Lauren A." last="Henderson">Lauren A. Henderson</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Hazen, Melissa M" sort="Hazen, Melissa M" uniqKey="Hazen M" first="Melissa M." last="Hazen">Melissa M. Hazen</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Bonfim, Carmem" sort="Bonfim, Carmem" uniqKey="Bonfim C" first="Carmem" last="Bonfim">Carmem Bonfim</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Wolska Ku Nierz, Beata" sort="Wolska Ku Nierz, Beata" uniqKey="Wolska Ku Nierz B" first="Beata" last="Wolska-Ku Nierz">Beata Wolska-Ku Nierz</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Butte, Manish J" sort="Butte, Manish J" uniqKey="Butte M" first="Manish J." last="Butte">Manish J. Butte</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Hernandez, Joseph D" sort="Hernandez, Joseph D" uniqKey="Hernandez J" first="Joseph D." last="Hernandez">Joseph D. Hernandez</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Nicholas, Sarah K" sort="Nicholas, Sarah K" uniqKey="Nicholas S" first="Sarah K." last="Nicholas">Sarah K. Nicholas</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Stepensky, Polina" sort="Stepensky, Polina" uniqKey="Stepensky P" first="Polina" last="Stepensky">Polina Stepensky</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Chandrakasan, Shanmuganathan" sort="Chandrakasan, Shanmuganathan" uniqKey="Chandrakasan S" first="Shanmuganathan" last="Chandrakasan">Shanmuganathan Chandrakasan</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Miano, Maurizio" sort="Miano, Maurizio" uniqKey="Miano M" first="Maurizio" last="Miano">Maurizio Miano</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Westermann Clark, Emma" sort="Westermann Clark, Emma" uniqKey="Westermann Clark E" first="Emma" last="Westermann-Clark">Emma Westermann-Clark</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Goda, Vera" sort="Goda, Vera" uniqKey="Goda V" first="Vera" last="Goda">Vera Goda</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Krivan, Gergely" sort="Krivan, Gergely" uniqKey="Krivan G" first="Gergely" last="Kriván">Gergely Kriván</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Holland, Steven M" sort="Holland, Steven M" uniqKey="Holland S" first="Steven M." last="Holland">Steven M. Holland</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Fadugba, Olajumoke" sort="Fadugba, Olajumoke" uniqKey="Fadugba O" first="Olajumoke" last="Fadugba">Olajumoke Fadugba</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Henrickson, Sarah E" sort="Henrickson, Sarah E" uniqKey="Henrickson S" first="Sarah E" last="Henrickson">Sarah E. Henrickson</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Ozen, Ahmet" sort="Ozen, Ahmet" uniqKey="Ozen A" first="Ahmet" last="Ozen">Ahmet Ozen</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Karakoc Aydiner, Elif" sort="Karakoc Aydiner, Elif" uniqKey="Karakoc Aydiner E" first="Elif" last="Karakoc-Aydiner">Elif Karakoc-Aydiner</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Baris, Safa" sort="Baris, Safa" uniqKey="Baris S" first="Safa" last="Baris">Safa Baris</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Kiykim, Ayca" sort="Kiykim, Ayca" uniqKey="Kiykim A" first="Ayca" last="Kiykim">Ayca Kiykim</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Bredius, Robbert" sort="Bredius, Robbert" uniqKey="Bredius R" first="Robbert" last="Bredius">Robbert Bredius</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Hoeger, Birgit" sort="Hoeger, Birgit" uniqKey="Hoeger B" first="Birgit" last="Hoeger">Birgit Hoeger</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Boztug, Kaan" sort="Boztug, Kaan" uniqKey="Boztug K" first="Kaan" last="Boztug">Kaan Boztug</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Pashchenko, Olga" sort="Pashchenko, Olga" uniqKey="Pashchenko O" first="Olga" last="Pashchenko">Olga Pashchenko</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Neven, Benedicte" sort="Neven, Benedicte" uniqKey="Neven B" first="Benedicte" last="Neven">Benedicte Neven</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Moshous, Despina" sort="Moshous, Despina" uniqKey="Moshous D" first="Despina" last="Moshous">Despina Moshous</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="De Villartay, Jean Pierre" sort="De Villartay, Jean Pierre" uniqKey="De Villartay J" first="Jean-Pierre" last="De Villartay">Jean-Pierre De Villartay</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Bousfiha, Ahmed Aziz" sort="Bousfiha, Ahmed Aziz" uniqKey="Bousfiha A" first="Ahmed Aziz" last="Bousfiha">Ahmed Aziz Bousfiha</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Hill, Harry R" sort="Hill, Harry R" uniqKey="Hill H" first="Harry R." last="Hill">Harry R. Hill</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Notarangelo, Luigi D" sort="Notarangelo, Luigi D" uniqKey="Notarangelo L" first="Luigi D." last="Notarangelo">Luigi D. Notarangelo</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Walter, Jolan E" sort="Walter, Jolan E" uniqKey="Walter J" first="Jolan E." last="Walter">Jolan E. Walter</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PMC</idno>
<idno type="pmid">30877075</idno>
<idno type="pmc">6612449</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6612449</idno>
<idno type="RBID">PMC:6612449</idno>
<idno type="doi">10.1016/j.jaip.2019.02.038</idno>
<date when="2019">2019</date>
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Outcomes and treatment strategies for autoimmunity and hyperinflammation in patients with RAG deficiency.</title>
<author><name sortKey="Farmer, Jocelyn R" sort="Farmer, Jocelyn R" uniqKey="Farmer J" first="Jocelyn R." last="Farmer">Jocelyn R. Farmer</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Foldvari, Zsofia" sort="Foldvari, Zsofia" uniqKey="Foldvari Z" first="Zsofia" last="Foldvari">Zsofia Foldvari</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Ujhazi, Boglarka" sort="Ujhazi, Boglarka" uniqKey="Ujhazi B" first="Boglarka" last="Ujhazi">Boglarka Ujhazi</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="De Ravin, Suk See" sort="De Ravin, Suk See" uniqKey="De Ravin S" first="Suk See" last="De Ravin">Suk See De Ravin</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Chen, Karin" sort="Chen, Karin" uniqKey="Chen K" first="Karin" last="Chen">Karin Chen</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Bleesing, Jack J H" sort="Bleesing, Jack J H" uniqKey="Bleesing J" first="Jack J. H." last="Bleesing">Jack J. H. Bleesing</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Schuetz, Catharina" sort="Schuetz, Catharina" uniqKey="Schuetz C" first="Catharina" last="Schuetz">Catharina Schuetz</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Al Herz, Waleed" sort="Al Herz, Waleed" uniqKey="Al Herz W" first="Waleed" last="Al-Herz">Waleed Al-Herz</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Abraham, Roshini Sarah" sort="Abraham, Roshini Sarah" uniqKey="Abraham R" first="Roshini Sarah" last="Abraham">Roshini Sarah Abraham</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Joshi, Avni Y" sort="Joshi, Avni Y" uniqKey="Joshi A" first="Avni Y." last="Joshi">Avni Y. Joshi</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Costa Carvalho, Beatriz T" sort="Costa Carvalho, Beatriz T" uniqKey="Costa Carvalho B" first="Beatriz T." last="Costa-Carvalho">Beatriz T. Costa-Carvalho</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Buchbinder, David" sort="Buchbinder, David" uniqKey="Buchbinder D" first="David" last="Buchbinder">David Buchbinder</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Booth, Claire" sort="Booth, Claire" uniqKey="Booth C" first="Claire" last="Booth">Claire Booth</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Reiff, Andreas" sort="Reiff, Andreas" uniqKey="Reiff A" first="Andreas" last="Reiff">Andreas Reiff</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Ferguson, Polly J" sort="Ferguson, Polly J" uniqKey="Ferguson P" first="Polly J." last="Ferguson">Polly J. Ferguson</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Aghamohammadi, Asghar" sort="Aghamohammadi, Asghar" uniqKey="Aghamohammadi A" first="Asghar" last="Aghamohammadi">Asghar Aghamohammadi</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Abolhassani, Hassan" sort="Abolhassani, Hassan" uniqKey="Abolhassani H" first="Hassan" last="Abolhassani">Hassan Abolhassani</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Puck, Jennifer M" sort="Puck, Jennifer M" uniqKey="Puck J" first="Jennifer M." last="Puck">Jennifer M. Puck</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Adeli, Mehdi" sort="Adeli, Mehdi" uniqKey="Adeli M" first="Mehdi" last="Adeli">Mehdi Adeli</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Cancrini, Caterina" sort="Cancrini, Caterina" uniqKey="Cancrini C" first="Caterina" last="Cancrini">Caterina Cancrini</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Palma, Paolo" sort="Palma, Paolo" uniqKey="Palma P" first="Paolo" last="Palma">Paolo Palma</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Bertaina, Alice" sort="Bertaina, Alice" uniqKey="Bertaina A" first="Alice" last="Bertaina">Alice Bertaina</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Locatelli, Franco" sort="Locatelli, Franco" uniqKey="Locatelli F" first="Franco" last="Locatelli">Franco Locatelli</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Di Matteo, Gigliola" sort="Di Matteo, Gigliola" uniqKey="Di Matteo G" first="Gigliola" last="Di Matteo">Gigliola Di Matteo</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Geha, Raif S" sort="Geha, Raif S" uniqKey="Geha R" first="Raif S." last="Geha">Raif S. Geha</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Kanariou, Maria G" sort="Kanariou, Maria G" uniqKey="Kanariou M" first="Maria G." last="Kanariou">Maria G. Kanariou</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Lycopoulou, Evangelia" sort="Lycopoulou, Evangelia" uniqKey="Lycopoulou E" first="Evangelia" last="Lycopoulou">Evangelia Lycopoulou</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Tzanoudaki, Marianna" sort="Tzanoudaki, Marianna" uniqKey="Tzanoudaki M" first="Marianna" last="Tzanoudaki">Marianna Tzanoudaki</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Sleasman, John W" sort="Sleasman, John W" uniqKey="Sleasman J" first="John W." last="Sleasman">John W. Sleasman</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Parikh, Suhag" sort="Parikh, Suhag" uniqKey="Parikh S" first="Suhag" last="Parikh">Suhag Parikh</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Pinero, Gloria" sort="Pinero, Gloria" uniqKey="Pinero G" first="Gloria" last="Pinero">Gloria Pinero</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Fischer, Bernard M" sort="Fischer, Bernard M" uniqKey="Fischer B" first="Bernard M." last="Fischer">Bernard M. Fischer</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Dbaibo, Ghassan" sort="Dbaibo, Ghassan" uniqKey="Dbaibo G" first="Ghassan" last="Dbaibo">Ghassan Dbaibo</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Unal, Ekrem" sort="Unal, Ekrem" uniqKey="Unal E" first="Ekrem" last="Unal">Ekrem Unal</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Patiroglu, Turkan" sort="Patiroglu, Turkan" uniqKey="Patiroglu T" first="Turkan" last="Patiroglu">Turkan Patiroglu</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Karakukcu, Musa" sort="Karakukcu, Musa" uniqKey="Karakukcu M" first="Musa" last="Karakukcu">Musa Karakukcu</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Al Saad, Khulood Khalifa" sort="Al Saad, Khulood Khalifa" uniqKey="Al Saad K" first="Khulood Khalifa" last="Al-Saad">Khulood Khalifa Al-Saad</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Dilley, Meredith A" sort="Dilley, Meredith A" uniqKey="Dilley M" first="Meredith A." last="Dilley">Meredith A. Dilley</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Pai, Sung Yun" sort="Pai, Sung Yun" uniqKey="Pai S" first="Sung-Yun" last="Pai">Sung-Yun Pai</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Dutmer, Cullen M" sort="Dutmer, Cullen M" uniqKey="Dutmer C" first="Cullen M." last="Dutmer">Cullen M. Dutmer</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Gelfand, Erwin W" sort="Gelfand, Erwin W" uniqKey="Gelfand E" first="Erwin W." last="Gelfand">Erwin W. Gelfand</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Geier, Christoph B" sort="Geier, Christoph B" uniqKey="Geier C" first="Christoph B." last="Geier">Christoph B. Geier</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Eibl, Martha M" sort="Eibl, Martha M" uniqKey="Eibl M" first="Martha M." last="Eibl">Martha M. Eibl</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Wolf, Hermann M" sort="Wolf, Hermann M" uniqKey="Wolf H" first="Hermann M." last="Wolf">Hermann M. Wolf</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Henderson, Lauren A" sort="Henderson, Lauren A" uniqKey="Henderson L" first="Lauren A." last="Henderson">Lauren A. Henderson</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Hazen, Melissa M" sort="Hazen, Melissa M" uniqKey="Hazen M" first="Melissa M." last="Hazen">Melissa M. Hazen</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Bonfim, Carmem" sort="Bonfim, Carmem" uniqKey="Bonfim C" first="Carmem" last="Bonfim">Carmem Bonfim</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Wolska Ku Nierz, Beata" sort="Wolska Ku Nierz, Beata" uniqKey="Wolska Ku Nierz B" first="Beata" last="Wolska-Ku Nierz">Beata Wolska-Ku Nierz</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Butte, Manish J" sort="Butte, Manish J" uniqKey="Butte M" first="Manish J." last="Butte">Manish J. Butte</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Hernandez, Joseph D" sort="Hernandez, Joseph D" uniqKey="Hernandez J" first="Joseph D." last="Hernandez">Joseph D. Hernandez</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Nicholas, Sarah K" sort="Nicholas, Sarah K" uniqKey="Nicholas S" first="Sarah K." last="Nicholas">Sarah K. Nicholas</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Stepensky, Polina" sort="Stepensky, Polina" uniqKey="Stepensky P" first="Polina" last="Stepensky">Polina Stepensky</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Chandrakasan, Shanmuganathan" sort="Chandrakasan, Shanmuganathan" uniqKey="Chandrakasan S" first="Shanmuganathan" last="Chandrakasan">Shanmuganathan Chandrakasan</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Miano, Maurizio" sort="Miano, Maurizio" uniqKey="Miano M" first="Maurizio" last="Miano">Maurizio Miano</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Westermann Clark, Emma" sort="Westermann Clark, Emma" uniqKey="Westermann Clark E" first="Emma" last="Westermann-Clark">Emma Westermann-Clark</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Goda, Vera" sort="Goda, Vera" uniqKey="Goda V" first="Vera" last="Goda">Vera Goda</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Krivan, Gergely" sort="Krivan, Gergely" uniqKey="Krivan G" first="Gergely" last="Kriván">Gergely Kriván</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Holland, Steven M" sort="Holland, Steven M" uniqKey="Holland S" first="Steven M." last="Holland">Steven M. Holland</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Fadugba, Olajumoke" sort="Fadugba, Olajumoke" uniqKey="Fadugba O" first="Olajumoke" last="Fadugba">Olajumoke Fadugba</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Henrickson, Sarah E" sort="Henrickson, Sarah E" uniqKey="Henrickson S" first="Sarah E" last="Henrickson">Sarah E. Henrickson</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Ozen, Ahmet" sort="Ozen, Ahmet" uniqKey="Ozen A" first="Ahmet" last="Ozen">Ahmet Ozen</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Karakoc Aydiner, Elif" sort="Karakoc Aydiner, Elif" uniqKey="Karakoc Aydiner E" first="Elif" last="Karakoc-Aydiner">Elif Karakoc-Aydiner</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Baris, Safa" sort="Baris, Safa" uniqKey="Baris S" first="Safa" last="Baris">Safa Baris</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Kiykim, Ayca" sort="Kiykim, Ayca" uniqKey="Kiykim A" first="Ayca" last="Kiykim">Ayca Kiykim</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Bredius, Robbert" sort="Bredius, Robbert" uniqKey="Bredius R" first="Robbert" last="Bredius">Robbert Bredius</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Hoeger, Birgit" sort="Hoeger, Birgit" uniqKey="Hoeger B" first="Birgit" last="Hoeger">Birgit Hoeger</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Boztug, Kaan" sort="Boztug, Kaan" uniqKey="Boztug K" first="Kaan" last="Boztug">Kaan Boztug</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Pashchenko, Olga" sort="Pashchenko, Olga" uniqKey="Pashchenko O" first="Olga" last="Pashchenko">Olga Pashchenko</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Neven, Benedicte" sort="Neven, Benedicte" uniqKey="Neven B" first="Benedicte" last="Neven">Benedicte Neven</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Moshous, Despina" sort="Moshous, Despina" uniqKey="Moshous D" first="Despina" last="Moshous">Despina Moshous</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="De Villartay, Jean Pierre" sort="De Villartay, Jean Pierre" uniqKey="De Villartay J" first="Jean-Pierre" last="De Villartay">Jean-Pierre De Villartay</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Bousfiha, Ahmed Aziz" sort="Bousfiha, Ahmed Aziz" uniqKey="Bousfiha A" first="Ahmed Aziz" last="Bousfiha">Ahmed Aziz Bousfiha</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Hill, Harry R" sort="Hill, Harry R" uniqKey="Hill H" first="Harry R." last="Hill">Harry R. Hill</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Notarangelo, Luigi D" sort="Notarangelo, Luigi D" uniqKey="Notarangelo L" first="Luigi D." last="Notarangelo">Luigi D. Notarangelo</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Walter, Jolan E" sort="Walter, Jolan E" uniqKey="Walter J" first="Jolan E." last="Walter">Jolan E. Walter</name>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
<affiliation><nlm:aff>NONE</nlm:aff>
</affiliation>
</author>
</analytic>
<series><title level="j">The journal of allergy and clinical immunology. In practice</title>
<idno type="ISSN">2213-2198</idno>
<idno type="eISSN">2213-2201</idno>
<imprint><date when="2019">2019</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent (MeSH)</term>
<term>Adult (MeSH)</term>
<term>Autoimmunity (MeSH)</term>
<term>Child (MeSH)</term>
<term>Child, Preschool (MeSH)</term>
<term>Female (MeSH)</term>
<term>Hematopoietic Stem Cell Transplantation (MeSH)</term>
<term>Homeodomain Proteins (MeSH)</term>
<term>Humans (MeSH)</term>
<term>Immunologic Deficiency Syndromes (immunology)</term>
<term>Immunologic Deficiency Syndromes (therapy)</term>
<term>Immunosuppressive Agents (therapeutic use)</term>
<term>Infant (MeSH)</term>
<term>Inflammation (MeSH)</term>
<term>Male (MeSH)</term>
<term>Middle Aged (MeSH)</term>
<term>Treatment Outcome (MeSH)</term>
<term>Young Adult (MeSH)</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Adolescent (MeSH)</term>
<term>Adulte (MeSH)</term>
<term>Adulte d'âge moyen (MeSH)</term>
<term>Auto-immunité (MeSH)</term>
<term>Déficits immunitaires (immunologie)</term>
<term>Déficits immunitaires (thérapie)</term>
<term>Enfant (MeSH)</term>
<term>Enfant d'âge préscolaire (MeSH)</term>
<term>Femelle (MeSH)</term>
<term>Humains (MeSH)</term>
<term>Immunosuppresseurs (usage thérapeutique)</term>
<term>Inflammation (MeSH)</term>
<term>Jeune adulte (MeSH)</term>
<term>Mâle (MeSH)</term>
<term>Nourrisson (MeSH)</term>
<term>Protéines à homéodomaine (MeSH)</term>
<term>Résultat thérapeutique (MeSH)</term>
<term>Transplantation de cellules souches hématopoïétiques (MeSH)</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Immunosuppressive Agents</term>
</keywords>
<keywords scheme="MESH" type="chemical" xml:lang="en"><term>Homeodomain Proteins</term>
</keywords>
<keywords scheme="MESH" qualifier="immunologie" xml:lang="fr"><term>Déficits immunitaires</term>
</keywords>
<keywords scheme="MESH" qualifier="immunology" xml:lang="en"><term>Immunologic Deficiency Syndromes</term>
</keywords>
<keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Immunologic Deficiency Syndromes</term>
</keywords>
<keywords scheme="MESH" qualifier="thérapie" xml:lang="fr"><term>Déficits immunitaires</term>
</keywords>
<keywords scheme="MESH" qualifier="usage thérapeutique" xml:lang="fr"><term>Immunosuppresseurs</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adolescent</term>
<term>Adult</term>
<term>Autoimmunity</term>
<term>Child</term>
<term>Child, Preschool</term>
<term>Female</term>
<term>Hematopoietic Stem Cell Transplantation</term>
<term>Humans</term>
<term>Infant</term>
<term>Inflammation</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Treatment Outcome</term>
<term>Young Adult</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Adolescent</term>
<term>Adulte</term>
<term>Adulte d'âge moyen</term>
<term>Auto-immunité</term>
<term>Enfant</term>
<term>Enfant d'âge préscolaire</term>
<term>Femelle</term>
<term>Humains</term>
<term>Inflammation</term>
<term>Jeune adulte</term>
<term>Mâle</term>
<term>Nourrisson</term>
<term>Protéines à homéodomaine</term>
<term>Résultat thérapeutique</term>
<term>Transplantation de cellules souches hématopoïétiques</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en"><sec id="S1"><title>Background:</title>
<p id="P5">While autoimmunity and hyperinflammation secondary to recombinase activating gene (RAG) deficiency have been associated with delayed diagnosis and even death, our current understanding is limited primarily to small case series.</p>
</sec>
<sec id="S2"><title>Objective:</title>
<p id="P6">Understand the frequency, severity, and treatment responsiveness of autoimmunity and hyperinflammation in RAG deficiency.</p>
</sec>
<sec id="S3"><title>Methods:</title>
<p id="P7">In reviewing the literature and our own database, we identified 85 patients with RAG deficiency, reported between 2001 and 2016, and compiled the largest case series to date of 63 patients with prominent autoimmune and/or hyperinflammatory pathology.</p>
</sec>
<sec id="S4"><title>Results:</title>
<p id="P8">Diagnosis of RAG deficiency was delayed a median of 5 years from the first clinical signs of immune dysregulation. The majority of patients (55.6%) presented with more than one autoimmune or hyperinflammatory complication, with the most common etiologies being cytopenias (84.1%), granulomas (23.8%), and inflammatory skin disorders (19.0%). Infections, including live viral vaccinations, closely preceded the onset of autoimmunity in 28.6% of cases. Autoimmune cytopenias had early onset (median 1.9, 2.1, and 2.6 years for autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP) and autoimmune neutropenia (AN), respectively) and were refractory to intravenous immunoglobulin, steroids, and rituximab in the majority of cases (64.7%, 73.7%, and 71.4% for AIHA, ITP, and AN, respectively). Evans syndrome specifically was associated with lack of response to first-line therapy. Treatment-refractory autoimmunity/hyperinflammation prompted hematopoietic stem cell transplantation in 20 patients.</p>
</sec>
<sec id="S5"><title>Conclusions:</title>
<p id="P9">Autoimmunity/hyperinflammation can be a presenting sign of RAG deficiency and should prompt further evaluation. Multi-lineage cytopenias are often refractory to immunosuppressive treatment and may require hematopoietic cell transplantation for definitive management.</p>
</sec>
</div>
</front>
</TEI>
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<name sortKey="Adeli, Mehdi" sort="Adeli, Mehdi" uniqKey="Adeli M" first="Mehdi" last="Adeli">Mehdi Adeli</name>
<name sortKey="Aghamohammadi, Asghar" sort="Aghamohammadi, Asghar" uniqKey="Aghamohammadi A" first="Asghar" last="Aghamohammadi">Asghar Aghamohammadi</name>
<name sortKey="Al Herz, Waleed" sort="Al Herz, Waleed" uniqKey="Al Herz W" first="Waleed" last="Al-Herz">Waleed Al-Herz</name>
<name sortKey="Al Saad, Khulood Khalifa" sort="Al Saad, Khulood Khalifa" uniqKey="Al Saad K" first="Khulood Khalifa" last="Al-Saad">Khulood Khalifa Al-Saad</name>
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<name sortKey="Notarangelo, Luigi D" sort="Notarangelo, Luigi D" uniqKey="Notarangelo L" first="Luigi D." last="Notarangelo">Luigi D. Notarangelo</name>
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Outcomes and treatment strategies for autoimmunity and hyperinflammation in patients with RAG deficiency.

Outcomes and treatment strategies for autoimmunity and hyperinflammation in patients with RAG deficiency.

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