<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>0864-0289</journal-id>
<journal-title><![CDATA[Revista Cubana de Hematología, Inmunología y Hemoterapia]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Cubana Hematol Inmunol Hemoter]]></abbrev-journal-title>
<issn>0864-0289</issn>
<publisher>
<publisher-name><![CDATA[Centro Nacional de Información de Ciencias MédicasEditorial Ciencias Médicas]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0864-02892021000400006</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Síndrome de Evans y desregulación inmune: complejidades moleculares y etiopatogénicas]]></article-title>
<article-title xml:lang="en"><![CDATA[Evans syndrome and immune dysregulation: molecular and etiopathogenic complexities]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Soler Noda]]></surname>
<given-names><![CDATA[Gilberto]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Escalona Muñoz]]></surname>
<given-names><![CDATA[Lilia Zenaida]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Instituto de Hematología e Inmunología  ]]></institution>
<addr-line><![CDATA[ La Habana]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad de Ciencias Médicas de La Habana Hospital Pediátrico Docente &#8220;William Soler&#8221; Facultad de Tecnología de la Salud]]></institution>
<addr-line><![CDATA[ La Habana]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2021</year>
</pub-date>
<volume>37</volume>
<numero>4</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S0864-02892021000400006&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S0864-02892021000400006&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S0864-02892021000400006&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  El síndrome de Evans se define como la presencia de citopenias inmunes que afectan dos o más líneas celulares simultánea o secuencialmente. Generalmente se refiere a la combinación de anemia hemolítica autoinmune con trombocitopenia inmune primaria, pero puede incluir también neutropenia autoinmune. Su etiología se atribuye a la producción de autoanticuerpos patológicos contra las células sanguíneas pero su causa real se desconoce. Objetivo: Explicar la relación del síndrome de Evans con la desregulación del sistema inmune. Método: Se realizó una revisión de la literatura en inglés y español a través del sitio web PubMed y el motor de búsqueda Google académico, de artículos publicados sobre el tema. El 69,73 % correspondieron a los últimos 5 años.  Conclusiones:  La inmunopatología del síndrome de Evans se puede atribuir a una alteración en el desarrollo o la función de los linfocitos, de manera que el equilibrio inmunológico se inclina hacia la autorreactividad.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  Evans syndrome is defined as the presence of autoimmune cytopenias affecting two or more blood cell lines, either simultaneously or sequentially. Most often, this refers to the combination of autoimmune hemolytic anemia and immune thrombocytopenia but can include autoimmune neutropenia as well. The etiology of Evans syndrome has been attributed to pathologic autoantibody production against the blood cells, but the true underlying cause remaining unknown.  Objective:  to explain the relationship of Evans syndrome with dysregulation of the immune system.  Method:  a review of the literature in English and Spanish was carried out through the PubMed website and the academic Google search engine for articles published on the subject. 69,73 % corresponded to the last 5 years.  Conclusions:  the immunopathology of Evans syndrome can be attributed to an alteration in the development or function of lymphocytes, such that the immune balance is inclined towards self-reactivity.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[síndrome de Evans]]></kwd>
<kwd lng="es"><![CDATA[lupus eritematoso sistémico]]></kwd>
<kwd lng="es"><![CDATA[síndrome linfoproliferativo autoinmune]]></kwd>
<kwd lng="es"><![CDATA[inmunodeficiencia variable común]]></kwd>
<kwd lng="en"><![CDATA[Evans syndrome]]></kwd>
<kwd lng="en"><![CDATA[systemic lupus erythematosus]]></kwd>
<kwd lng="en"><![CDATA[autoimmune lymphoproliferative syndrome]]></kwd>
<kwd lng="en"><![CDATA[common variable immunodeficiency]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Al Ghaithi]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Wright]]></surname>
<given-names><![CDATA[NAM]]></given-names>
</name>
<name>
<surname><![CDATA[Breakey]]></surname>
<given-names><![CDATA[VR]]></given-names>
</name>
<name>
<surname><![CDATA[Cox]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Warias]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Wong]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Combined autoimmune cytopenias presenting in childhood]]></article-title>
<source><![CDATA[Pediatr Blood Cancer]]></source>
<year>2016</year>
<volume>63</volume>
<page-range>292-8</page-range></nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mantadakis]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Farmaki]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Natural history, pathogenesis, and treatment of Evans syndrome in children]]></article-title>
<source><![CDATA[J Pediatr Hematol Oncol]]></source>
<year>2017</year>
<volume>39</volume>
<page-range>413-9</page-range></nlm-citation>
</ref>
<ref id="B3">
<label>3</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Michel]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Chanet]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Dechartres]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Morin]]></surname>
<given-names><![CDATA[AS]]></given-names>
</name>
<name>
<surname><![CDATA[Piette]]></surname>
<given-names><![CDATA[JC]]></given-names>
</name>
<name>
<surname><![CDATA[Cirasino]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The spectrum of Evans syndrome in adults new insight into the disease based on the analysis of 68 cases]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2009</year>
<volume>114</volume>
<numero>15</numero>
<issue>15</issue>
<page-range>3167-72</page-range></nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sipurzynski]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Fahrner]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Kerbl]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Crazzolara]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Jones]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Ebetsberger]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Management of chronic immune thrombocytopenia in children and adolescents lessons from an Austrian national cross-sectional study of 81 patients]]></article-title>
<source><![CDATA[Semin Hematol]]></source>
<year>2016</year>
<volume>53</volume>
<page-range>S43-7</page-range></nlm-citation>
</ref>
<ref id="B5">
<label>5</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Yong]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Schoonen]]></surname>
<given-names><![CDATA[WM]]></given-names>
</name>
<name>
<surname><![CDATA[Li]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Kanas]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Coalson]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Mowat]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Epidemiology of paediatric immune thrombocytopenia in the General Practice Research Database]]></article-title>
<source><![CDATA[Br J Haematol]]></source>
<year>2010</year>
<volume>149</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>855-64</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Aladjidi]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Jutand]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
<name>
<surname><![CDATA[Beaubois]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Fernandes]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Jeanpetit]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Coureau]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Reliable assessment of the incidence of childhood autoimmune hemolytic anemia]]></article-title>
<source><![CDATA[Pediatr Blood Cancer]]></source>
<year>2017</year>
<volume>64</volume>
</nlm-citation>
</ref>
<ref id="B7">
<label>7</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Olmsted]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Despotovic]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Primary and Secondary Immune Cytopenias Evaluation and Treatment Approach in Children]]></article-title>
<source><![CDATA[Hematol Oncol Clin N Am]]></source>
<year>2019</year>
<volume>33</volume>
<page-range>489-506</page-range></nlm-citation>
</ref>
<ref id="B8">
<label>8</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hadjadj]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Aladjidi]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Fernandes]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Leverger]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Magerus-Chatinet]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Mazerolles]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Pediatric Evans syndrome is associated with a high frequency of potentially damaging variants in immune genes]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2019</year>
<volume>134</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>9-21</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sipurzynski]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Fahrner]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Kerbl]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Crazzolara]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Jones]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Ebetsberger]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Management of chronic immune thrombocytopenia in children and adolescents lessons from an Austrian national cross-sectional study of 81 patients]]></article-title>
<source><![CDATA[Semin Hematol]]></source>
<year>2016</year>
<volume>53</volume>
<numero>^s1</numero>
<issue>^s1</issue>
<supplement>1</supplement>
<page-range>S43-7</page-range></nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Norton]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Roberts]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Management of Evans syndrome]]></article-title>
<source><![CDATA[B J Haematol]]></source>
<year>2006</year>
<volume>132</volume>
<page-range>125-37</page-range></nlm-citation>
</ref>
<ref id="B11">
<label>11</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Aladjidi]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Leverger]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Leblanc]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Quitterie Picat]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Michel]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Bertrand]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[New insights into childhood autoimmune hemolytic anemia a French national observational study of 265 children]]></article-title>
<source><![CDATA[Haematologica]]></source>
<year>2011</year>
<volume>96</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>655-63</page-range></nlm-citation>
</ref>
<ref id="B12">
<label>12</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Bride]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Teachey]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Autoimmune lymphoproliferative syndrome more than a FAScinating disease]]></article-title>
<source><![CDATA[F1000Res]]></source>
<year>2017</year>
<volume>6</volume>
<page-range>1928</page-range></nlm-citation>
</ref>
<ref id="B13">
<label>13</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Schmidt]]></surname>
<given-names><![CDATA[RE]]></given-names>
</name>
<name>
<surname><![CDATA[Grimbacher]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Witte]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Autoimmunity and primary immunodeficiency two sides of the same coin?]]></article-title>
<source><![CDATA[Nat Rev Rheumatol]]></source>
<year>2018</year>
<volume>14</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>7-18</page-range></nlm-citation>
</ref>
<ref id="B14">
<label>14</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Seidel]]></surname>
<given-names><![CDATA[MG]]></given-names>
</name>
<name>
<surname><![CDATA[Kindle]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Gathmann]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Quinti]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Buckland]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[van Montfrans]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The European Society for Immunodeficiencies (ESID) Registry Working Definitions for the Clinical Diagnosis of Inborn Errors of Immunity]]></article-title>
<source><![CDATA[J Allergy Clin Immunol Pract]]></source>
<year>2019</year>
<volume>7</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>17631-70</page-range></nlm-citation>
</ref>
<ref id="B15">
<label>15</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[NijmanI]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[van Montfrans]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
<name>
<surname><![CDATA[Hoogstraat]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Boes]]></surname>
<given-names><![CDATA[ML]]></given-names>
</name>
<name>
<surname><![CDATA[van de Corput]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Renner]]></surname>
<given-names><![CDATA[ED]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Targeted next-generation sequencing: a novel diagnostic tool for primary immunodeficiencies]]></article-title>
<source><![CDATA[J Allergy Clin Immunol]]></source>
<year>2014</year>
<volume>133</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>529-34</page-range></nlm-citation>
</ref>
<ref id="B16">
<label>16</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Li]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Grimes]]></surname>
<given-names><![CDATA[AB]]></given-names>
</name>
<name>
<surname><![CDATA[Rider]]></surname>
<given-names><![CDATA[NL]]></given-names>
</name>
<name>
<surname><![CDATA[Mahoney]]></surname>
<given-names><![CDATA[DH]]></given-names>
</name>
<name>
<surname><![CDATA[Fleisher]]></surname>
<given-names><![CDATA[TA]]></given-names>
</name>
<name>
<surname><![CDATA[Shearer]]></surname>
<given-names><![CDATA[WT]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Diagnostic dilemma ALPS versus Evans syndrome]]></article-title>
<source><![CDATA[Clin Immunol]]></source>
<year>2017</year>
<volume>183</volume>
<page-range>247-8</page-range></nlm-citation>
</ref>
<ref id="B17">
<label>17</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Zhang]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Wu]]></surname>
<given-names><![CDATA[X]]></given-names>
</name>
<name>
<surname><![CDATA[Wang]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Li]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Chen]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Zhao]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical features of systemic lupus erythematosus patients complicated with Evans syndrome a case-control, single center study]]></article-title>
<source><![CDATA[Medicine (Baltimore)]]></source>
<year>2016</year>
<volume>95</volume>
<numero>15</numero>
<issue>15</issue>
</nlm-citation>
</ref>
<ref id="B18">
<label>18</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Patel B]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Jakob]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A Rare Case of Simultaneous Evans Syndrome and Primary Antiphospholipid Syndrome]]></article-title>
<source><![CDATA[Cureus]]></source>
<year>2020</year>
<volume>12</volume>
<numero>2</numero>
<issue>2</issue>
</nlm-citation>
</ref>
<ref id="B19">
<label>19</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Matson]]></surname>
<given-names><![CDATA[DR]]></given-names>
</name>
<name>
<surname><![CDATA[Yang]]></surname>
<given-names><![CDATA[DT]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Autoimmune Lymphoproliferative Syndrome An Overview]]></article-title>
<source><![CDATA[Arch Pathol Lab Med]]></source>
<year>2020</year>
<volume>144</volume>
<page-range>245-51</page-range></nlm-citation>
</ref>
<ref id="B20">
<label>20</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Xie]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Pittaluga]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Price]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Raffeld]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Hahn]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Jaffe]]></surname>
<given-names><![CDATA[ES]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Bone marrow findings in autoimmune lymphoproliferative syndrome with germline FAS mutation]]></article-title>
<source><![CDATA[Haematologica]]></source>
<year>2017</year>
<volume>102</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>364-72</page-range></nlm-citation>
</ref>
<ref id="B21">
<label>21</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Seif]]></surname>
<given-names><![CDATA[AE]]></given-names>
</name>
<name>
<surname><![CDATA[Manno]]></surname>
<given-names><![CDATA[CS]]></given-names>
</name>
<name>
<surname><![CDATA[Sheen]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Grupp]]></surname>
<given-names><![CDATA[SA]]></given-names>
</name>
<name>
<surname><![CDATA[Teachey]]></surname>
<given-names><![CDATA[DT]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Identifying autoimmune lymphoproliferative syndrome in children with Evans syndrome: a multi-institutional study]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2010</year>
<volume>115</volume>
<numero>11</numero>
<issue>11</issue>
<page-range>2142-5</page-range></nlm-citation>
</ref>
<ref id="B22">
<label>22</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Oliveira]]></surname>
<given-names><![CDATA[JB]]></given-names>
</name>
<name>
<surname><![CDATA[Bleesing]]></surname>
<given-names><![CDATA[JJ]]></given-names>
</name>
<name>
<surname><![CDATA[Dianzani]]></surname>
<given-names><![CDATA[U]]></given-names>
</name>
<name>
<surname><![CDATA[Fleisher]]></surname>
<given-names><![CDATA[TA]]></given-names>
</name>
<name>
<surname><![CDATA[Jaffe]]></surname>
<given-names><![CDATA[ES]]></given-names>
</name>
<name>
<surname><![CDATA[Lenardo]]></surname>
<given-names><![CDATA[MJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS) report from the 2009 NIH International Workshop]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2010</year>
<volume>116</volume>
<numero>14</numero>
<issue>14</issue>
<page-range>e35-40</page-range></nlm-citation>
</ref>
<ref id="B23">
<label>23</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Ho]]></surname>
<given-names><![CDATA[HE]]></given-names>
</name>
<name>
<surname><![CDATA[Cunningham-Rundles]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Non-infectious Complications of Common Variable Immunodeficiency Updated Clinical Spectrum, Sequelae, and Insights to Pathogenesis]]></article-title>
<source><![CDATA[Front Immunol]]></source>
<year>2020</year>
<volume>11</volume>
<page-range>149</page-range></nlm-citation>
</ref>
<ref id="B24">
<label>24</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Feuille]]></surname>
<given-names><![CDATA[EJ]]></given-names>
</name>
<name>
<surname><![CDATA[Anooshiravani]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Sullivan]]></surname>
<given-names><![CDATA[KE]]></given-names>
</name>
<name>
<surname><![CDATA[Fuleihan]]></surname>
<given-names><![CDATA[RL]]></given-names>
</name>
<name>
<surname><![CDATA[Cunningham-Rundles]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Autoimmune cytopenias and associated conditions in CVID a report from the USIDNET Registry]]></article-title>
<source><![CDATA[J Clin Immunol]]></source>
<year>2017</year>
<volume>38</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>28-34</page-range></nlm-citation>
</ref>
<ref id="B25">
<label>25</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lube]]></surname>
<given-names><![CDATA[GE]]></given-names>
</name>
<name>
<surname><![CDATA[Ferriani]]></surname>
<given-names><![CDATA[MP]]></given-names>
</name>
<name>
<surname><![CDATA[Campos]]></surname>
<given-names><![CDATA[LM]]></given-names>
</name>
<name>
<surname><![CDATA[Terreri]]></surname>
<given-names><![CDATA[MT]]></given-names>
</name>
<name>
<surname><![CDATA[Bonfá]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Saad]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Evans syndrome at childhood-onset systemic lupus erythematosus diagnosis a large multicenter study]]></article-title>
<source><![CDATA[Pediatr Blood Cancer]]></source>
<year>2016</year>
<volume>63</volume>
<numero>7</numero>
<issue>7</issue>
<page-range>1238-43</page-range></nlm-citation>
</ref>
<ref id="B26">
<label>26</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Burnett]]></surname>
<given-names><![CDATA[DL]]></given-names>
</name>
<name>
<surname><![CDATA[Langley]]></surname>
<given-names><![CDATA[DB]]></given-names>
</name>
<name>
<surname><![CDATA[Schofield]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Hermes]]></surname>
<given-names><![CDATA[JR]]></given-names>
</name>
<name>
<surname><![CDATA[Chan]]></surname>
<given-names><![CDATA[TD]]></given-names>
</name>
<name>
<surname><![CDATA[Jackson]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Germinal center antibody mutation trajectories are determined by rapid self/foreign discrimination]]></article-title>
<source><![CDATA[Science]]></source>
<year>2018</year>
<volume>360</volume>
<numero>6385</numero>
<issue>6385</issue>
<page-range>223-6</page-range></nlm-citation>
</ref>
<ref id="B27">
<label>27</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Warnatz]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Wehr]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Dräger]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Schmidt]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Eibel]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Schlesier]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Expansion of CD19(hi)CD21(lo/neg) B cells in common variable immunodeficiency (CVID) patients with autoimmune cytopenia]]></article-title>
<source><![CDATA[Immunobiology]]></source>
<year>2002</year>
<volume>206</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>502-13</page-range></nlm-citation>
</ref>
<ref id="B28">
<label>28</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Romberg]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Le Coz]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Glauzy]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Schickel]]></surname>
<given-names><![CDATA[JN]]></given-names>
</name>
<name>
<surname><![CDATA[TrofaBA]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Nolan]]></surname>
<given-names><![CDATA[BE]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Patients with common variable immunodeficiency with autoimmune cytopenias exhibit hyperplastic yet inefficient germinal center responses]]></article-title>
<source><![CDATA[J Allergy Clin Immunol]]></source>
<year>2019</year>
<volume>143</volume>
<page-range>258</page-range></nlm-citation>
</ref>
<ref id="B29">
<label>29</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kuwana]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Okazaki]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Kajihara]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Kaburaki]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Miyazaki]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Kawakami]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Autoantibody to c-Mpl (thrombopoietin receptor) in systemic lupus erythematosus relationship to thrombocytopenia with megakaryocytic hypoplasia]]></article-title>
<source><![CDATA[Arthritis Rheum]]></source>
<year>2002</year>
<volume>46</volume>
<page-range>2148-59</page-range></nlm-citation>
</ref>
<ref id="B30">
<label>30</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Cines]]></surname>
<given-names><![CDATA[DB]]></given-names>
</name>
<name>
<surname><![CDATA[Liebman]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Stasi]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Pathobiology of secondary immune thrombocytopenia]]></article-title>
<source><![CDATA[Semin Hematol]]></source>
<year>2009</year>
<volume>46</volume>
<numero>1^s2</numero>
<issue>1^s2</issue>
<supplement>2</supplement>
<page-range>S2-14</page-range></nlm-citation>
</ref>
<ref id="B31">
<label>31</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Nakamura]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Tanaka]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Satoh]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Kawai]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Hirakata]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Kaburaki]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Autoantibody to CD40 ligand in systemic lupus erythematosus association with thrombocytopenia but not thromboembolism]]></article-title>
<source><![CDATA[Rheumatology (Oxford)]]></source>
<year>2006</year>
<volume>45</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>150-6</page-range></nlm-citation>
</ref>
<ref id="B32">
<label>32</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Liu]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Chen]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Sun]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Lin]]></surname>
<given-names><![CDATA[Q]]></given-names>
</name>
<name>
<surname><![CDATA[Liao]]></surname>
<given-names><![CDATA[X]]></given-names>
</name>
<name>
<surname><![CDATA[Zhang]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical characteristics of immune thrombocytopenia associated with autoimmune disease a retrospective study]]></article-title>
<source><![CDATA[Medicine (Baltimore)]]></source>
<year>2016</year>
<volume>95</volume>
<numero>50</numero>
<issue>50</issue>
</nlm-citation>
</ref>
<ref id="B33">
<label>33</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Molnár]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Radwan]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Kovács]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Andrikovics]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Henriquez]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Zarafov]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Key diagnostic markers for Autoimmune Lymphoproliferative Syndrome with molecular genetic diagnosis]]></article-title>
<source><![CDATA[Blood blood]]></source>
<year></year>
</nlm-citation>
</ref>
<ref id="B34">
<label>34</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Takagi]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Hoshino]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Yoshida]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Ueno]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Imai]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Piao]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Genetic heterogeneity of uncharacterized childhood autoimmune diseases with lymphoproliferation]]></article-title>
<source><![CDATA[Pediatr Blood Cancer]]></source>
<year>2018</year>
<volume>65</volume>
</nlm-citation>
</ref>
<ref id="B35">
<label>35</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Li]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Huang]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Yang]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Hao]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Peng]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Li]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Updated Understanding of Autoimmune Lymphoproliferative Syndrome (ALPS)]]></article-title>
<source><![CDATA[Clinic Rev Allerg Immunol]]></source>
<year></year>
</nlm-citation>
</ref>
<ref id="B36">
<label>36</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kuehn]]></surname>
<given-names><![CDATA[HS]]></given-names>
</name>
<name>
<surname><![CDATA[Caminha]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Niemela]]></surname>
<given-names><![CDATA[JE]]></given-names>
</name>
<name>
<surname><![CDATA[Rao]]></surname>
<given-names><![CDATA[VK]]></given-names>
</name>
<name>
<surname><![CDATA[Davis]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Fleisher]]></surname>
<given-names><![CDATA[TA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[FAS haploinsuficiency is a common disease mechanism in the human autoimmune lymphoproliferative syndrome]]></article-title>
<source><![CDATA[J Immunol]]></source>
<year>2011</year>
<volume>186</volume>
<numero>10</numero>
<issue>10</issue>
<page-range>6035-43</page-range></nlm-citation>
</ref>
<ref id="B37">
<label>37</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Magerus-Chatinet]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Neven]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Stolzenberg]]></surname>
<given-names><![CDATA[MC]]></given-names>
</name>
<name>
<surname><![CDATA[Daussy]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Arkwright]]></surname>
<given-names><![CDATA[PD]]></given-names>
</name>
<name>
<surname><![CDATA[Lanzarotti]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Onset of autoimmune lymphoproliferative syndrome (ALPS) in humans as a consequence of genetic defect accumulation]]></article-title>
<source><![CDATA[J Clin Invest]]></source>
<year>2011</year>
<volume>121</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>106-12</page-range></nlm-citation>
</ref>
<ref id="B38">
<label>38</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Boggio]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Gigliotti]]></surname>
<given-names><![CDATA[CL]]></given-names>
</name>
<name>
<surname><![CDATA[Rossi]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Toffoletti]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Cappellano]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Clemente]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Decreased function of Fas and variations of the perforin gene in adult patients with primary immune thrombocytopenia]]></article-title>
<source><![CDATA[B J Haematol]]></source>
<year>2017</year>
<volume>176</volume>
<page-range>258-67</page-range></nlm-citation>
</ref>
<ref id="B39">
<label>39</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[van Schouwenburg]]></surname>
<given-names><![CDATA[PA]]></given-names>
</name>
<name>
<surname><![CDATA[IJspeert]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Pico-Knijnenburg]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Dalm]]></surname>
<given-names><![CDATA[VASH]]></given-names>
</name>
<name>
<surname><![CDATA[van Hagen]]></surname>
<given-names><![CDATA[PM]]></given-names>
</name>
<name>
<surname><![CDATA[van Zessen]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Identification of CVID Patients With Defects in Immune Repertoire Formation or Specification]]></article-title>
<source><![CDATA[Front Immunol]]></source>
<year>2018</year>
<volume>9</volume>
<page-range>2545</page-range></nlm-citation>
</ref>
<ref id="B40">
<label>40</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Odnoletkova]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Kindle]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Quinti]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Grimbacher]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Knerr]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Gathmann]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The burden of common variable immunodeficiency disorders a retrospective analysis of the European Society for Immunodeficiency (ESID) registry data]]></article-title>
<source><![CDATA[Orphanet J Rare Dis]]></source>
<year>2018</year>
<volume>13</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>201</page-range></nlm-citation>
</ref>
<ref id="B41">
<label>41</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Yazdani]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Seify]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Ganjalikhani-Hakemi]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Abolhassani]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Eskandari]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Golsaz-Shirazi]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Comparison of various classifications for patients with common variable immunodeficiency (CVID) using measurement of B-cell subsets]]></article-title>
<source><![CDATA[Allergol Immunopathol (Madr)]]></source>
<year>2017</year>
<volume>45</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>183-92</page-range></nlm-citation>
</ref>
<ref id="B42">
<label>42</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Meffre]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The establishment of early B cell tolerance in humans lessons from primary immunodeiciency diseases]]></article-title>
<source><![CDATA[Ann N Y Acad Sci]]></source>
<year>2011</year>
<volume>1246</volume>
<page-range>1-10</page-range></nlm-citation>
</ref>
<ref id="B43">
<label>43</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Yazdani]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Habibi]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Sharifi]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Azizi]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Abolhassani]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Olbrich]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Common variable immunodeficiency epidemiology, pathogenesis, clinical manifestations, diagnosis, classification and management]]></article-title>
<source><![CDATA[J Investig Allergol Clin Immunol]]></source>
<year>2020</year>
<volume>30</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>14-34</page-range></nlm-citation>
</ref>
<ref id="B44">
<label>44</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Takagi]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Ogata]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Ueno]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Yoshida]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Yeh]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Hoshino]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Haploinsufficiency of TNFAIP3 (A20) by germline mutation is involved in autoimmune lymphoproliferative syndrome]]></article-title>
<source><![CDATA[J Allergy Clin I mmunol]]></source>
<year>2017</year>
<volume>139</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>1914-22</page-range></nlm-citation>
</ref>
<ref id="B45">
<label>45</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Yakaboski]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Fuleihan]]></surname>
<given-names><![CDATA[RL]]></given-names>
</name>
<name>
<surname><![CDATA[Sullivan]]></surname>
<given-names><![CDATA[KE]]></given-names>
</name>
<name>
<surname><![CDATA[Cunningham-Rundles]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Feuille]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Lymphoproliferative Disease in CVID A Report of Types and Frequencies from a US Patient Registry]]></article-title>
<source><![CDATA[J Clin Immunol]]></source>
<year>2020</year>
<volume>40</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>524-30</page-range></nlm-citation>
</ref>
<ref id="B46">
<label>46</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Oliveira]]></surname>
<given-names><![CDATA[JB]]></given-names>
</name>
<name>
<surname><![CDATA[Bidère]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Niemela]]></surname>
<given-names><![CDATA[JE]]></given-names>
</name>
<name>
<surname><![CDATA[Zheng]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Sakai]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Nix]]></surname>
<given-names><![CDATA[CP]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[NRAS mutation causes a human autoimmune lymphoproliferative syndrome]]></article-title>
<source><![CDATA[Proc Natl Acad Sci U S A]]></source>
<year>2007</year>
<volume>104</volume>
<numero>21</numero>
<issue>21</issue>
<page-range>8953-8</page-range></nlm-citation>
</ref>
<ref id="B47">
<label>47</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Niemela]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Lu]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Fleisher]]></surname>
<given-names><![CDATA[TA]]></given-names>
</name>
<name>
<surname><![CDATA[Davis]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Caminha]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Natter]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Somatic KRAS mutations associated with a human nonmalignant syndrome of autoimmunity and abnormal leukocyte homeostasis]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2011</year>
<volume>117</volume>
<page-range>2883-6</page-range></nlm-citation>
</ref>
<ref id="B48">
<label>48</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Chun]]></surname>
<given-names><![CDATA[HJ]]></given-names>
</name>
<name>
<surname><![CDATA[Zheng]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Ahmad]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Wang]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Speirs]]></surname>
<given-names><![CDATA[CK]]></given-names>
</name>
<name>
<surname><![CDATA[Siegel]]></surname>
<given-names><![CDATA[RM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Pleiotropic defects in lymphocyte activation caused by caspase-8 mutations lead to human immunodeiciency]]></article-title>
<source><![CDATA[Nature]]></source>
<year>2002</year>
<volume>419</volume>
<numero>6905</numero>
<issue>6905</issue>
<page-range>395-9</page-range></nlm-citation>
</ref>
<ref id="B49">
<label>49</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Shiota]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Yang]]></surname>
<given-names><![CDATA[X]]></given-names>
</name>
<name>
<surname><![CDATA[Kubokawa]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Morishima]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Tanaka]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Mikami]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Somatic mosaicism for a NRAS mutation associates with disparate clinical features in RAS-associated leukoproliferative disease a report of two cases]]></article-title>
<source><![CDATA[J Clin Immunol]]></source>
<year>2015</year>
<volume>35</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>454-8</page-range></nlm-citation>
</ref>
<ref id="B50">
<label>50</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Niemela]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Lu]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Fleisher]]></surname>
<given-names><![CDATA[TA]]></given-names>
</name>
<name>
<surname><![CDATA[Davis]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Caminha]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Natter]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Somatic KRAS mutations associated with a human nonmalignant syndrome of autoimmunity and abnormal leukocyte homeostasis]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2011</year>
<volume>117</volume>
<page-range>2883-6</page-range></nlm-citation>
</ref>
<ref id="B51">
<label>51</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kucuk]]></surname>
<given-names><![CDATA[ZY]]></given-names>
</name>
<name>
<surname><![CDATA[Charbonnier]]></surname>
<given-names><![CDATA[LM]]></given-names>
</name>
<name>
<surname><![CDATA[McMasters]]></surname>
<given-names><![CDATA[RL]]></given-names>
</name>
<name>
<surname><![CDATA[Chatila]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Bleesing]]></surname>
<given-names><![CDATA[JJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[CTLA-4 haploinsuficiency in a patient with an autoimmune lymphoproliferative disorder]]></article-title>
<source><![CDATA[J Allergy Clin Immunol]]></source>
<year>2017</year>
<volume>140</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>862-4</page-range></nlm-citation>
</ref>
<ref id="B52">
<label>52</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Gámez-Díaz]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[August]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Stepensky]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Revel-Vilk]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Seidel]]></surname>
<given-names><![CDATA[MG]]></given-names>
</name>
<name>
<surname><![CDATA[Noriko]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The extended phenotype of LPS-responsive beige-like anchor protein (LRBA) deficiency]]></article-title>
<source><![CDATA[J Allergy Clin Immunol]]></source>
<year>2016</year>
<volume>137</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>223-30</page-range></nlm-citation>
</ref>
<ref id="B53">
<label>53</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kostel Bal]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Haskologlu]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Serwas]]></surname>
<given-names><![CDATA[NK]]></given-names>
</name>
<name>
<surname><![CDATA[Islamoglu]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Aytekin]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Kendirli]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Multiple presentations of LRBA deficiency a single-center experience]]></article-title>
<collab>et al</collab>
<source><![CDATA[J Clin Immunol]]></source>
<year>2017</year>
<volume>37</volume>
<numero>8</numero>
<issue>8</issue>
<page-range>790-800</page-range></nlm-citation>
</ref>
<ref id="B54">
<label>54</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Coulter]]></surname>
<given-names><![CDATA[TI]]></given-names>
</name>
<name>
<surname><![CDATA[Chandra]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Bacon]]></surname>
<given-names><![CDATA[CM]]></given-names>
</name>
<name>
<surname><![CDATA[Babar]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Curtis]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Screaton]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical spectrum and features of activated phosphoinositide 3-kinase d syndrome a large patient cohort study]]></article-title>
<source><![CDATA[J Allergy Clin Immunol]]></source>
<year>2017</year>
<volume>139</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>597-606</page-range></nlm-citation>
</ref>
<ref id="B55">
<label>55</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Consonni]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Dotta]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Todaro]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Vairo]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Badolato]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Signal transducer and activator of transcription gain-of-function primary immunodeficiency/immunodysregulation disorders]]></article-title>
<source><![CDATA[Curr Opin Pediatr]]></source>
<year>2017</year>
<volume>29</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>711-7</page-range></nlm-citation>
</ref>
<ref id="B56">
<label>56</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Schwab]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Gabrysch]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Olbrich]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Patiño]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Warnatz]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Wolff]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Phenotype, penetrance, and treatment of 133 cytotoxic T-lymphocyte antigen 4-insufficient subjects]]></article-title>
<source><![CDATA[J Allergy Clin Immunol]]></source>
<year>2018</year>
<volume>142</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>1932-46</page-range></nlm-citation>
</ref>
<ref id="B57">
<label>57</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lo]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Fritz]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
<name>
<surname><![CDATA[Su]]></surname>
<given-names><![CDATA[HC]]></given-names>
</name>
<name>
<surname><![CDATA[Uzel]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Jordan]]></surname>
<given-names><![CDATA[MB]]></given-names>
</name>
<name>
<surname><![CDATA[Lenardo]]></surname>
<given-names><![CDATA[MJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[CHAI and LATAIE new genetic diseases of CTLA-4 checkpoint insufficiency]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2016</year>
<volume>128</volume>
<page-range>1037-42</page-range></nlm-citation>
</ref>
<ref id="B58">
<label>58</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lévy]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Stolzenberg]]></surname>
<given-names><![CDATA[MC]]></given-names>
</name>
<name>
<surname><![CDATA[Bruneau]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Breton]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Neven]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Sauvion]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[LRBA deficiency with autoimmunity and early onset chronic erosive polyarthritis]]></article-title>
<source><![CDATA[Clin Immunol]]></source>
<year>2016</year>
<volume>168</volume>
<page-range>88-93</page-range></nlm-citation>
</ref>
<ref id="B59">
<label>59</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Azizi]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Abolhassani]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Zaki-Dizaji]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Habibi]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Mohammadi]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Shaghaghi]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Polyautoimmunity in patients with LPS-responsive beige-like anchor (LRBA) deficiency]]></article-title>
<source><![CDATA[Immunol Investig]]></source>
<year>2018</year>
<volume>47</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>457-67</page-range></nlm-citation>
</ref>
<ref id="B60">
<label>60</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Besnard]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Levy]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Aladjidi]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Stolzenberg]]></surname>
<given-names><![CDATA[MC]]></given-names>
</name>
<name>
<surname><![CDATA[Magerus-Chatinet]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Alibeu]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Pediatric-onset Evans syndrome Heterogeneous presentation and high frequency of monogenic disorders including LRBA and CTLA4 mutations]]></article-title>
<source><![CDATA[Clin Immunol]]></source>
<year>2018</year>
<volume>188</volume>
<page-range>52-7</page-range></nlm-citation>
</ref>
<ref id="B61">
<label>61</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Coulter]]></surname>
<given-names><![CDATA[TI]]></given-names>
</name>
<name>
<surname><![CDATA[Chandra]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Bacon]]></surname>
<given-names><![CDATA[CM]]></given-names>
</name>
<name>
<surname><![CDATA[Babar]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Curtis]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Screaton]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical spectrum and features of activated phosphoinositide 3-kinase d syndrome a large patient cohort study]]></article-title>
<source><![CDATA[J Allergy Clin Immunol]]></source>
<year>2017</year>
<volume>139</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>597-606</page-range></nlm-citation>
</ref>
<ref id="B62">
<label>62</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Volkl]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Rensing-Ehl]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Allgauer]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Schreiner]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Lorenz]]></surname>
<given-names><![CDATA[MR]]></given-names>
</name>
<name>
<surname><![CDATA[Rohr]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hyperactive mTOR pathway promotes lymphoproliferation and abnormal differentiation in autoimmune lymphoproliferative syndrome]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2016</year>
<volume>128</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>227-38</page-range></nlm-citation>
</ref>
<ref id="B63">
<label>63</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Preite]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Gomez-Rodriguez]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Cannons]]></surname>
<given-names><![CDATA[JL]]></given-names>
</name>
<name>
<surname><![CDATA[Schwartzberg]]></surname>
<given-names><![CDATA[PL]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[T and B-cell signaling in activated PI3K delta syndrome From immunodeficiency to autoimmunity]]></article-title>
<source><![CDATA[Immunol Rev]]></source>
<year>2019</year>
<volume>291</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>154-73</page-range></nlm-citation>
</ref>
<ref id="B64">
<label>64</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Klemann]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Esquivel]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Magerus-Chatinet]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Lorenz]]></surname>
<given-names><![CDATA[MR]]></given-names>
</name>
<name>
<surname><![CDATA[Fuchs]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Neveux]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Evolution of disease activity and biomarkers on and off rapamycin in 28 patients with autoimmune lymphoproliferative syndrome]]></article-title>
<source><![CDATA[Haematologica]]></source>
<year>2017</year>
<volume>102</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>e52-6</page-range></nlm-citation>
</ref>
<ref id="B65">
<label>65</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Thauland]]></surname>
<given-names><![CDATA[TJ]]></given-names>
</name>
<name>
<surname><![CDATA[Pellerin]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Ohgami]]></surname>
<given-names><![CDATA[RS]]></given-names>
</name>
<name>
<surname><![CDATA[Bacchetta]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Butte]]></surname>
<given-names><![CDATA[MJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Case Study Mechanism for Increased Follicular Helper T Cell Development in Activated PI3K Delta Syndrome]]></article-title>
<source><![CDATA[Front Immunol]]></source>
<year>2019</year>
<volume>10</volume>
<page-range>753</page-range></nlm-citation>
</ref>
<ref id="B66">
<label>66</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Coulter]]></surname>
<given-names><![CDATA[TI]]></given-names>
</name>
<name>
<surname><![CDATA[Chandra]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Bacon]]></surname>
<given-names><![CDATA[CM]]></given-names>
</name>
<name>
<surname><![CDATA[Babar]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Curtis]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Screaton]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical spectrum and features of activated phosphoinositide 3-kinase d syndrome a large patient cohort study]]></article-title>
<source><![CDATA[J Allergy Clin Immunol]]></source>
<year>2017</year>
<volume>139</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>597-606</page-range></nlm-citation>
</ref>
<ref id="B67">
<label>67</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Consonni]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Dotta]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Todaro]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Vairo]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Badolato]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Signal transducer and activator of transcription gain-of-function primary immunodeficiency/immunodysregulation disorders]]></article-title>
<source><![CDATA[Curr Opin Pediatr]]></source>
<year>2017</year>
<volume>29</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>711-7</page-range></nlm-citation>
</ref>
<ref id="B68">
<label>68</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Weinacht]]></surname>
<given-names><![CDATA[KG]]></given-names>
</name>
<name>
<surname><![CDATA[Charbonnier]]></surname>
<given-names><![CDATA[LM]]></given-names>
</name>
<name>
<surname><![CDATA[Alrogi]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Plant]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Qiao]]></surname>
<given-names><![CDATA[Q]]></given-names>
</name>
<name>
<surname><![CDATA[Wu]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Ruxolitinib reverses dysregulated T helper cell responses and controls autoimmunity caused by a novel signal transducer and activator of transcription 1 (STAT1) gain-of-function mutation]]></article-title>
<source><![CDATA[J Allergy Clin Immunol]]></source>
<year>2017</year>
<volume>139</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>1629-40</page-range></nlm-citation>
</ref>
<ref id="B69">
<label>69</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Forbes]]></surname>
<given-names><![CDATA[LR]]></given-names>
</name>
<name>
<surname><![CDATA[Milner]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Haddad]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Signal transducer and activator of transcription 3 a year in review]]></article-title>
<source><![CDATA[Curr Opin Hematol]]></source>
<year>2016</year>
<volume>23</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>23-7</page-range></nlm-citation>
</ref>
<ref id="B70">
<label>70</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Nabhani]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Schipp]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Miskin]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Levin]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Postovsky]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Dujovny]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[STAT3 gain-of-function mutations associated with autoimmune lymphoproliferative syndrome like disease deregulate lymphocyte apoptosis and can be targeted by BH3 mimetic compounds]]></article-title>
<collab>et al</collab>
<source><![CDATA[Clin Immunol]]></source>
<year>2017</year>
<volume>181</volume>
<page-range>32-42</page-range></nlm-citation>
</ref>
<ref id="B71">
<label>71</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Schafiq]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Schipp]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Miskin]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Levin]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Postovsky]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Dujovny]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[STAT3 gain-of-function mutations associated with autoimmune lymphoproliferative syndrome like disease deregulate lymphocyte apoptosis and can be targeted by BH3 mimetic compounds]]></article-title>
<source><![CDATA[Clin Immunol]]></source>
<year>2017</year>
<volume>181</volume>
<page-range>32-42</page-range></nlm-citation>
</ref>
<ref id="B72">
<label>72</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Yakaboski]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Fuleihan]]></surname>
<given-names><![CDATA[RL]]></given-names>
</name>
<name>
<surname><![CDATA[Sullivan]]></surname>
<given-names><![CDATA[KE]]></given-names>
</name>
<name>
<surname><![CDATA[Cunningham-Rundles]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Feuille]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Lymphoproliferative Disease in CVID A Report of Types and Frequencies from a US Patient Registry]]></article-title>
<source><![CDATA[J Clin Immunol]]></source>
<year>2020</year>
<volume>40</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>524-30</page-range></nlm-citation>
</ref>
<ref id="B73">
<label>73</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Bleesing]]></surname>
<given-names><![CDATA[JJ]]></given-names>
</name>
<name>
<surname><![CDATA[Brown]]></surname>
<given-names><![CDATA[MR]]></given-names>
</name>
<name>
<surname><![CDATA[Straus]]></surname>
<given-names><![CDATA[SE]]></given-names>
</name>
<name>
<surname><![CDATA[Dale]]></surname>
<given-names><![CDATA[JK]]></given-names>
</name>
<name>
<surname><![CDATA[Siegel]]></surname>
<given-names><![CDATA[RM]]></given-names>
</name>
<name>
<surname><![CDATA[Johnson]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Immunophenotypic profiles in families with autoimmune lymphoproliferative syndrome]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2001</year>
<volume>98</volume>
<numero>8</numero>
<issue>8</issue>
<page-range>2466-73</page-range></nlm-citation>
</ref>
<ref id="B74">
<label>74</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Caminha]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Fleisher]]></surname>
<given-names><![CDATA[TA]]></given-names>
</name>
<name>
<surname><![CDATA[Hornung]]></surname>
<given-names><![CDATA[RL]]></given-names>
</name>
<name>
<surname><![CDATA[Dale]]></surname>
<given-names><![CDATA[JK]]></given-names>
</name>
<name>
<surname><![CDATA[Niemela]]></surname>
<given-names><![CDATA[JE]]></given-names>
</name>
<name>
<surname><![CDATA[Price]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Using biomarkers to predict the presence of FAS mutations in patients with features of the autoimmune lymphoproliferative syndrome]]></article-title>
<source><![CDATA[J Allergy Clin Immunol]]></source>
<year>2010</year>
<volume>125</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>946-9</page-range></nlm-citation>
</ref>
<ref id="B75">
<label>75</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Maglione]]></surname>
<given-names><![CDATA[PJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Autoimmune and lymphoproliferative complications of common variable immunodeiciency]]></article-title>
<source><![CDATA[Curr Allergy Asthma Rep]]></source>
<year>2016</year>
<volume>16</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>19</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
