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<meta name="keywords" content="C4025672, cell or molecular dysfunction, impaired memory b cell generation, autosomal dominant, autosomal recessive, birth defects, chromosomal disease, chromosome, clinical features, clinical findings, clinical genetics, clinical recommendations, clinvar, congenital chromosomal disease, consumer genetic resources, cytogenetic location, disease characteristics, disease definitions, disease descriptions, disease ontology, disease synonyms, disease vocabulary, dysmorphology, entrez, familial disease, gene, gene-disease relationship, genereviews, genetic disease, genetic disorder, genetic terminology, genetic testing registry, genetics home reference, genomic disease, gtr, hereditary disease, heritable disease, hpo, human phenotype ontology, inherited disease, management guidelines, maternal inheritance, medgen, medical genetics, medical subject headings, mesh, mitochondrial inheritance, mode of inheritance, national center for biotechnology information, national institutes of health, national library of medicine, ncbi, nih, nlm, omim, ordo, orphanet, paternal inheritance, phenome, position statements, professional practice guidelines, rare disease, reference sequence, refseq, snomed ct, syndrome, undiagnosed diseases, x-linked recessive" /><meta name="description" content="Impaired production of memory cells, the B cells that persist for years or an entire lifetime and which confer rapid and enhanced response to secondary challenge." /><meta name="robots" content="index,nofollow,noarchive" />
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<!--
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||
UID=871195
|
||
ConceptID=C4025672
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-->
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||
<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Impaired memory B cell generation</div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>871195</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C4025672</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Cell or Molecular Dysfunction</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>HPO:</td>
|
||
<td><a target="_blank" title="Human Phenotype Ontology" href="https://hpo.jax.org/app/browse/term/HP:0002847">HP:0002847</a></td></tr>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Definition">Definition</h1><a sid="100" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln">Impaired production of memory cells, the B cells that persist for years or an entire lifetime and which confer rapid and enhanced response to secondary challenge. [from <a title="Human Phenotype Ontology" href="http://www.human-phenotype-ontology.org" class="defSource" target="_blank">HPO</a>]</div>
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<div class="portlet mgSection" id="ID_118">
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Term_Hierarchy">Term Hierarchy</h1><a sid="118" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
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<div class="portlet_content ln HierarchyGTR"><div class="jig-ncbitabs"><ul><li><a href="#tabGTR">GTR</a></li><li><a href="#tabMGEN">MeSH</a></li></ul><div id="tabGTR"><div class="search_result"><div class="rprts"><div class="chiclet_legend"><span class="chiclet_list" style="position:static;"><span title="Clinical test" class="chiclet Ccolor round">C</span><span>Clinical test, </span><span title="Research test" class="chiclet Rcolor round">R</span><span>Research test, </span><span title="OMIM" class="chiclet Ocolor ">O</span><span>OMIM, </span><span title="GeneReview" class="chiclet Gcolor">G</span><span><em>GeneReviews</em>, </span><span title="ClinVar" class="chiclet Vcolor">V</span><span>ClinVar </span></span></div><div id="hierarchy" class="margin_t1"><div class="ds_tree"><ul><li class="matched_ds"><span class="chiclet_list"><span class="chiclet unavailable round" title="Clinical test">C</span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet unavailable" title="OMIM">O</span><span class="chiclet unavailable" title="GeneReviews">G</span><span class="chiclet unavailable" title="ClinVar">V</span></span><span class="TLline">Impaired memory B cell generation</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/867443" ref="tree=MeSH" title="MedGen record for Phenotypic abnormality">Phenotypic abnormality</a></span><ul><li><span class="TLline"><a href="/medgen/867388" ref="tree=MeSH" title="MedGen record for Abnormality of the immune system">Abnormality of the immune system</a></span><ul><li><span class="TLline"><a href="/medgen/869194" ref="tree=MeSH" title="MedGen record for Abnormality of immune system physiology">Abnormality of immune system physiology</a></span><ul><li><span class="TLline"><a href="/medgen/1780121" ref="tree=MeSH" title="MedGen record for Abnormal leukocyte physiology">Abnormal leukocyte physiology</a></span><ul><li><span class="TLline"><a href="/medgen/1627345" ref="tree=MeSH" title="MedGen record for Abnormal lymphocyte physiology">Abnormal lymphocyte physiology</a></span><ul><li><span class="TLline"><a href="/medgen/341411" ref="tree=MeSH" title="MedGen record for Abnormality of B cell physiology">Abnormality of B cell physiology</a></span><ul><li><span class="matched_ds">Impaired memory B cell generation</span></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></div></div></div></div>
|
||
</div>
|
||
|
||
<div class="portlet mgSection" id="ID_112">
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||
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Conditions_with_this_feature">Conditions with this feature</h1><a sid="112" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln clinfeat">
|
||
<div class="divPopper rprt" id="rdis_96019"><div><strong>Hyper-IgM syndrome type 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>96019</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information."><span class="highlight" style="background-color:">C0398689</span></a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">X-linked hyper IgM syndrome (HIGM1), a disorder of abnormal T- and B-cell function, is characterized by low serum concentrations of IgG, IgA, and IgE with normal or elevated serum concentrations of IgM. Mitogen proliferation may be normal, but NK- and T-cell cytotoxicity can be impaired. Antigen-specific responses are usually decreased or absent. Total numbers of B cells are normal but there is a marked reduction of class-switched memory B cells. Defective oxidative burst of both neutrophils and macrophages has been reported. The range of clinical findings varies, even within the same family. More than 50% of males with HIGM1 develop symptoms by age one year, and more than 90% are symptomatic by age four years. HIGM1 usually presents in infancy with recurrent upper- and lower-respiratory tract bacterial infections, opportunistic infections including Pneumocystis jirovecii pneumonia, and recurrent or protracted diarrhea that can be infectious or noninfectious and is associated with failure to thrive. Neutropenia is common; thrombocytopenia and anemia are less commonly seen. Autoimmune and/or inflammatory disorders (such as sclerosing cholangitis) as well as increased risk for neoplasms have been reported as medical complications of this disorder. Significant neurologic complications, often the result of a CNS infection, are seen in 5%-15% of affected males. Liver disease, a serious complication of HIGM1 once observed in more than 80% of affected males by age 20 years, may be decreasing with adequate screening and treatment of Cryptosporidium infection.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/96019">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_328419"><div><strong>Hyper-IgM syndrome type 3</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>328419</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C1720957</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Type 3 immunodeficiency with hyper-IgM (HIGM3), first described in humans by Ferrari et al. (2001), is characterized by hypogammaglobulinemia with normal or elevated levels of IgM. For a general phenotypic description and a discussion of genetic heterogeneity of immunodeficiency with hyper-IgM, see HIGM1 (308230).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/328419">Condition Record</a></div></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_96019" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Hyper-IgM syndrome type 1</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_328419" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Hyper-IgM syndrome type 3</a></div></div>
|
||
</div>
|
||
<div class="display-none help-popup" id="guidelinesHelpPM">These guidelines are articles in PubMed that match specific search criteria developed by MedGen to capture the most relevant practice guidelines. This list may not be comprehensive and may include broader topics as well. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div><div class="display-none help-popup" id="guidelinesHelpCurated">These guidelines are manually curated by the MedGen team
|
||
to supplement articles available in PubMed. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div>
|
||
<div class="portlet mgSection" id="ID_103">
|
||
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_clinical_studies">Recent clinical studies</h1><a sid="103" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln"><h3 class="subhead">Etiology</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/39049686">Anti-TNF therapy impairs both short- and long-term IgG responses after repeated vaccination.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Buhre JS,
|
||
Pongracz T,
|
||
Geisen UM,
|
||
Schubert M,
|
||
Wang W,
|
||
Nouta J,
|
||
Obara M,
|
||
Lehrian S,
|
||
Rahmöller J,
|
||
Petry J,
|
||
Tran F,
|
||
Schreiber S,
|
||
Sümbül M,
|
||
Berner D,
|
||
Gerdes S,
|
||
Schirmer J,
|
||
Longardt AC,
|
||
Hoff P,
|
||
Kalinke U,
|
||
Ludwig RJ,
|
||
Bartsch YC,
|
||
Hoyer BF,
|
||
Wuhrer M,
|
||
Ehlers M</span><br />
|
||
<span class="medgenPMjournal">Allergy</span>
|
||
2025 Feb;80(2):423-439.
|
||
Epub 2024 Jul 25
|
||
doi: 10.1111/all.16241.
|
||
<span class="bold">PMID: </span><a href="/pubmed/39049686" target="_blank">39049686</a><a href="/pmc/articles/PMC11804311" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/39254005">Genomic insights into mRNA COVID-19 vaccines efficacy: Linking genetic polymorphisms to waning immunity.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Hsieh MJ,
|
||
Tsai PH,
|
||
Chiang PH,
|
||
Kao ZK,
|
||
Zhuang ZQ,
|
||
Hsieh AR,
|
||
Ho HL,
|
||
Chiou SH,
|
||
Liang KH,
|
||
Chen YC</span><br />
|
||
<span class="medgenPMjournal">Hum Vaccin Immunother</span>
|
||
2024 Dec 31;20(1):2399382.
|
||
Epub 2024 Sep 10
|
||
doi: 10.1080/21645515.2024.2399382.
|
||
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Alsina L,
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Allende LM,
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Gonzalez-Granado LI,
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Böhler C,
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Thiel J,
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Venhoff N,
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Lorenzetti R,
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Warnatz K,
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Unger S,
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Seidl M,
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Mielenz D,
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Schneider P,
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Ehl S,
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Rensing-Ehl A,
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Smulski CR,
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Rizzi M</span><br />
|
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<span class="medgenPMjournal">Sci Immunol</span>
|
||
2024 Jan 12;9(91):eadj5948.
|
||
doi: 10.1126/sciimmunol.adj5948.
|
||
<span class="bold">PMID: </span><a href="/pubmed/38215192" target="_blank">38215192</a></div>
|
||
|
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<div class="nl"><a target="_blank" href="/pubmed/34220839">Short- and Long-Lived Autoantibody-Secreting Cells in Autoimmune Neurological Disorders.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Zografou C,
|
||
Vakrakou AG,
|
||
Stathopoulos P</span><br />
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||
<span class="medgenPMjournal">Front Immunol</span>
|
||
2021;12:686466.
|
||
Epub 2021 Jun 17
|
||
doi: 10.3389/fimmu.2021.686466.
|
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<span class="bold">PMID: </span><a href="/pubmed/34220839" target="_blank">34220839</a><a href="/pmc/articles/PMC8248361" target="_blank" class="PubMedFree">Free PMC Article</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/33333111">The Immune Dysregulation of Common Variable Immunodeficiency Disorders.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Fernando SL,
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Jang HS,
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Li J</span><br />
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||
<span class="medgenPMjournal">Immunol Lett</span>
|
||
2021 Feb;230:21-26.
|
||
Epub 2020 Dec 14
|
||
doi: 10.1016/j.imlet.2020.12.002.
|
||
<span class="bold">PMID: </span><a href="/pubmed/33333111" target="_blank">33333111</a></div>
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