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<!--
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UID=163750
|
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ConceptID=C0876991
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-->
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<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Hemophagocytosis</div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>163750</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.">C0876991</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonym:</td>
|
||
<td>Hemocytophagia</td></tr>
|
||
<tr><td><span class="bold">SNOMED CT: </span></td>
|
||
<td>Hemophagocytosis (61070002); Hemocytophagia (61070002)</td></tr>
|
||
<tr><td colspan="2" class="small"> </td></tr><tr><td>HPO:</td>
|
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<td><a target="_blank" title="Human Phenotype Ontology" href="https://hpo.jax.org/app/browse/term/HP:0012156">HP:0012156</a></td></tr>
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<div class="portlet mgSection" id="ID_100">
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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">Phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors in bone marrow and other tissues. [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="TLline">Hemophagocytosis</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><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/1702861" ref="tree=MeSH" title="MedGen record for Abnormal immune system morphology">Abnormal immune system morphology</a></span><ul><li><span class="TLline"><a href="/medgen/869190" ref="tree=MeSH" title="MedGen record for Abnormal cellular immune system morphology">Abnormal cellular immune system morphology</a></span><ul><li><span class="TLline"><a href="/medgen/508852" ref="tree=MeSH" title="MedGen record for Abnormal leukocyte morphology">Abnormal leukocyte morphology</a></span><ul><li><span class="TLline"><a href="/medgen/869196" ref="tree=MeSH" title="MedGen record for Abnormal myeloid leukocyte morphology">Abnormal myeloid leukocyte morphology</a></span><ul><li><span class="TLline"><a href="/medgen/867298" ref="tree=MeSH" title="MedGen record for Abnormal macrophage morphology">Abnormal macrophage morphology</a></span><ul><li><span class="matched_ds">Hemophagocytosis</span><ul><li><span class="TLline"><a href="/medgen/1054305" ref="tree=MeSH" title="MedGen record for Bone marrow hemophagocytosis">Bone marrow hemophagocytosis</a></span></li><li><span class="TLline"><a href="/medgen/1842108" ref="tree=MeSH" title="MedGen record for CSF hemophagocytosis">CSF hemophagocytosis</a></span></li><li><span class="TLline"><a href="/medgen/86219" ref="tree=MeSH" title="MedGen record for Erythrophagocytosis">Erythrophagocytosis</a></span></li><li><span class="TLline"><a href="/medgen/1842040" ref="tree=MeSH" title="MedGen record for Hepatic hemophagocytosis">Hepatic hemophagocytosis</a></span></li><li><span class="TLline"><a href="/medgen/1842112" ref="tree=MeSH" title="MedGen record for Lymph-node hemophagocytosis">Lymph-node hemophagocytosis</a></span></li><li><span class="TLline"><a href="/medgen/1841575" ref="tree=MeSH" title="MedGen record for Splenic hemophagocytosis">Splenic hemophagocytosis</a></span></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></div></div></div></div>
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<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_7929"><div><strong>Primary myelofibrosis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>7929</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.">C0001815</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Neoplastic Process</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Primary myelofibrosis is a condition characterized by the buildup of scar tissue (fibrosis) in the bone marrow, the tissue that produces blood cells. Because of the fibrosis, the bone marrow is unable to make enough normal blood cells. The shortage of blood cells causes many of the signs and symptoms of primary myelofibrosis.\n\nInitially, most people with primary myelofibrosis have no signs or symptoms. Eventually, fibrosis can lead to a reduction in the number of red blood cells, white blood cells, and platelets. A shortage of red blood cells (anemia) often causes extreme tiredness (fatigue) or shortness of breath. A loss of white blood cells can lead to an increased number of infections, and a reduction of platelets can cause easy bleeding or bruising.\n\nBecause blood cell formation (hematopoiesis) in the bone marrow is disrupted, other organs such as the spleen or liver may begin to produce blood cells. This process, called extramedullary hematopoiesis, often leads to an enlarged spleen (splenomegaly) or an enlarged liver (hepatomegaly). People with splenomegaly may feel pain or fullness in the abdomen, especially below the ribs on the left side. Other common signs and symptoms of primary myelofibrosis include fever, night sweats, and bone pain.\n\nPrimary myelofibrosis is most commonly diagnosed in people aged 50 to 80 but can occur at any age.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/7929">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_3347"><div><strong>Chédiak-Higashi syndrome</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>3347</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:">C0007965</span></a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Chediak-Higashi syndrome (CHS) is characterized by partial oculocutaneous albinism (OCA), immunodeficiency, a mild bleeding tendency, and late adolescent- to adult-onset neurologic manifestations (e.g., learning difficulties, peripheral neuropathy, ataxia, and parkinsonism). While present in nearly all individuals with CHS, these clinical findings vary in severity. Of note, all individuals with CHS are at risk of developing neurologic manifestations and hemophagocytic lymphohistiocytosis (HLH). Individuals with severe childhood-onset presentations are considered to have "classic" CHS, whereas individuals with milder adolescent- to adult-onset presentations are considered to have "atypical" CHS. Because of the considerable overlap between classic CHS and atypical CHS, the disorder is best understood as a continuum of severe to milder phenotypes, with the universal feature being the pathognomonic giant granules within leukocytes observed on peripheral blood smear.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/3347">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_75704"><div><strong>Lysinuric protein intolerance</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>75704</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.">C0268647</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Lysinuric protein intolerance (LPI) typically presents after an infant is weaned from breast milk or formula; variable findings include recurrent vomiting and episodes of diarrhea, episodes of stupor and coma after a protein-rich meal, poor feeding, aversion to protein-rich food, failure to thrive, hepatosplenomegaly, and muscular hypotonia. Over time, findings include: poor growth, osteoporosis, involvement of the lungs (progressive interstitial changes, pulmonary alveolar proteinosis) and of the kidneys (progressive glomerular and proximal tubular disease), hematologic abnormalities (normochromic or hypochromic anemia, leukopenia, thrombocytopenia, erythroblastophagocytosis in the bone marrow aspirate), and a clinical presentation resembling the hemophagocytic lymphohistiocytosis/macrophagic activation syndrome. Hypercholesterolemia, hypertriglyceridemia, and acute pancreatitis can also be seen.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/75704">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_99306"><div><strong>Subcutaneous panniculitis-like T-cell lymphoma</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>99306</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.">C0522624</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Neoplastic Process</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon form of T-cell non-Hodgkin lymphoma in which cytotoxic CD8 (see 186910)+ T cells infiltrate adipose tissue forming subcutaneous nodules. Both children and adults can be affected, with a median age at diagnosis of 36 years and a female gender bias. Most patients have accompanying systemic features such as fever or flank pain. A subset (about 20%) of patients develop hemophagocytic lymphohistiocytosis (HLH), usually associated with CD8+ T cells rimming adipocytes in the bone marrow. An infectious agent is not identified, and the disorder is believed to result from improperly activated inflammation. Immunosuppressive therapy may be helpful; hematopoietic bone marrow transplantation is usually curative (summary by Gayden et al., 2018). For a general discussion of genetic heterogeneity of HLH, see HLH1 (267700).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/99306">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_332383"><div><strong>Familial hemophagocytic lymphohistiocytosis 3</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>332383</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.">C1837174</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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||
<div class="spaceAbove">Secretion of the contents of cytolytic granules at the immunologic synapse is a highly regulated process essential for lymphocyte cytotoxicity. This process requires the rapid transfer of perforin (170280)-containing lytic granules to the target cell interface, followed by their docking and fusion with the plasma membrane. Familial hemophagocytic lymphohistiocytosis is a genetically heterogeneous condition characterized by defective cytotoxicity. For a more detailed description of FHL, see 267700.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/332383">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_336848"><div><strong>X-linked lymphoproliferative disease due to XIAP deficiency</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>336848</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.">C1845076</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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||
<div class="spaceAbove">X-linked lymphoproliferative disease (XLP) in general is characterized by an inappropriate immune response to Epstein-Barr virus (EBV) infection leading to hemophagocytic lymphohistiocytosis (HLH) or severe mononucleosis, dysgammaglobulinemia, and lymphoproliferative disease (malignant lymphoma). The condition primarily affects males. XLP has two recognizable subtypes, XLP1 (due to pathogenic variants in SH2D1A) and XLP2 (due to pathogenic variants in XIAP). HLH / fulminant infectious mononucleosis is the most common presentation regardless of subtype. HLH is characterized as an acute illness with prolonged and high fever, bi- or trilineage cytopenias, and hepatosplenomegaly, which is often severe or fatal. Death is generally secondary to liver failure or multisystem organ dysfunction. In those with XLP1, dys- or hypogammaglobulinemia can lead to varying degrees of humoral immune dysfunction associated with bronchiectasis and recurrent respiratory infections that, if untreated, may result in death. Lymphoproliferative disease (malignant lymphoma) and other lymphoproliferative diseases are specific to XLP1 and often develop in childhood, usually following EBV exposure. Rarer findings in those with XLP1 can include aplastic anemia, vasculitis, and lymphoid granulomatosis. Males with XLP2 are more likely to have HLH without EBV infection, recurrent episodes of HLH (which is not typically seen in those with XLP1), splenomegaly, and gastrointestinal disease, including enterocolitis and perirectal abscesses or fistulae. Rarely, individuals with XLP2 and inflammatory bowel disease have been reported to develop inflammatory liver disease, which can progress to fatal liver failure. Transient hypogammaglobulinemia has been rarely observed in those with XLP2. To date, neither lymphoproliferative disease nor common variable immunodeficiency has been reported in males with XLP2. Heterozygous females rarely have symptoms. There are, however, increasing numbers of reports of affected females with unfavorable (skewed) X-chromosome inactivation favoring the X chromosome with the pathogenic variant who develop HLH, inflammatory bowel disease, and erythema nodosum.</div>
|
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<div class="spaceAbove nowrap">See: <a href="/medgen/336848">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_400366"><div><strong>Familial hemophagocytic lymphohistiocytosis 2</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>400366</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.">C1863727</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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||
<div class="spaceAbove">Familial hemophagocytic lymphohistiocytosis-2 (FHL2) is an autosomal recessive disorder of immune dysregulation with onset in infancy or early childhood. It is characterized clinically by fever, edema, hepatosplenomegaly, and liver dysfunction. Neurologic impairment, seizures, and ataxia are frequent. Laboratory studies show pancytopenia, coagulation abnormalities, hypofibrinogenemia, and hypertriglyceridemia. There is increased production of cytokines, such as gamma-interferon (IFNG; 147570) and TNF-alpha (191160), by hyperactivation and proliferation of T cells and macrophages. Activity of cytotoxic T cells and NK cells is reduced, consistent with a defect in cellular cytotoxicity. Bone marrow, lymph nodes, spleen, and liver show features of hemophagocytosis. Chemotherapy and/or immunosuppressant therapy may result in symptomatic remission, but the disorder is fatal without bone marrow transplantation (summary by Dufourcq-Lagelouse et al., 1999, Stepp et al., 1999, and Molleran Lee et al., 2004). For a general phenotypic description and a discussion of genetic heterogeneity of FHL, see 267700.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/400366">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_350245"><div><strong>Familial hemophagocytic lymphohistiocytosis 4</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>350245</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.">C1863728</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Hemophagocytic lymphohistiocytosis is a hyperinflammatory disorder clinically diagnosed based on the fulfillment of 5 of 8 criteria, including fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, hemophagocytosis, low or absent natural killer (NK) cell activity, hyperferritinemia, and high soluble IL2 receptor levels (IL2R; 147730). The disorder typically presents in infancy or early childhood. Persistent remission is rarely achieved with chemo- or immunotherapy; hematopoietic stem cell transplantation is the only cure (summary by Muller et al., 2014). For a phenotypic description and a discussion of genetic heterogeneity of familial hemophagocytic lymphohistiocytosis (FHL), see 267700.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/350245">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_357030"><div><strong>Griscelli syndrome type 2</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>357030</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.">C1868679</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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||
<div class="spaceAbove">Griscelli syndrome type 2 (GS2) is an autosomal recessive disorder characterized by pigmentary dilution of the skin and hair, the presence of large clumps of pigment in hair shafts, and an accumulation of melanosomes in melanocytes. Patients also have immunologic abnormalities with or without neurologic impairment (summary by Menasche et al., 2000). Some GS2 patients have been reported in whom central nervous system manifestations are the first presentation (Rajadhyax et al., 2007, Masri et al., 2008; Mishra et al., 2014; Lee et al., 2017). For a discussion of phenotypic and genetic heterogeneity of Griscelli syndrome, see Griscelli syndrome type 1 (GS1; 214450).</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/357030">Condition Record</a></div></div>
|
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<div class="divPopper rprt" id="rdis_416514"><div><strong>Familial hemophagocytic lymphohistiocytosis 5</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>416514</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.">C2751293</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Familial hemophagocytic lymphohistiocytosis-5 with or without microvillus inclusion disease (FHL5) is an autosomal recessive hyperinflammatory disorder characterized clinically by fever, hepatosplenomegaly, pancytopenia, coagulation abnormalities, and other laboratory findings. Some patients have neurologic symptoms due to inflammatory CNS disease. There is uncontrolled and ineffective proliferation and activation of T lymphocytes, NK cells, and macrophages that infiltrate multiple organs, including liver, spleen, lymph nodes, and the CNS. The phenotype is variable: some patients may present in early infancy with severe diarrhea, prior to the onset of typical FHL features, whereas others present later in childhood and have a more protracted course without diarrhea. The early-onset diarrhea is due to enteropathy reminiscent of microvillus inclusion disease (see MVID, 251850). The enteropathy, which often necessitates parenteral feeding, may be the most life-threatening issue even after hematopoietic stem cell transplantation (HSCT). More variable features include sensorineural hearing loss and hypogammaglobulinemia. Treatment with immunosuppressive drugs and chemotherapy can ameliorate signs and symptoms of FHL in some patients, but the only curative therapy for FHL is HSCT. HSCT is not curative for enteropathy associated with the disorder, despite hematologic and immunologic reconstitution (summary by Meeths et al., 2010; Pagel et al., 2012; Stepensky et al., 2013). For a phenotypic description and a discussion of genetic heterogeneity of familial hemophagocytic lymphohistiocytosis (FHL, HLH), see 267700.</div>
|
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<div class="spaceAbove nowrap">See: <a href="/medgen/416514">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_765548"><div><strong>Lymphoproliferative syndrome 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>765548</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.">C3552634</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Lymphoproliferative syndrome-1 is an autosomal recessive primary immunodeficiency characterized by onset in early childhood of Epstein-Barr virus (EBV)-associated immune dysregulation, manifest as lymphoma, lymphomatoid granulomatosis, hemophagocytic lymphohistiocytosis, Hodgkin disease, and/or hypogammaglobulinemia. Autoimmune disorders, such as autoimmune hemolytic anemia or renal disease, may also occur. Patients show a high EBV viral load and decreased invariant natural killer T cells. It is unknown whether patients with ITK mutations are intrinsically susceptible to development of lymphoma or dysgammaglobulinemia in the absence of EBV infection (summary by Stepensky et al., 2011; Linka et al., 2012). For a discussion of genetic heterogeneity of lymphoproliferative syndrome, see XLP1 (308240).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/765548">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_767454"><div><strong>Lymphoproliferative syndrome 2</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>767454</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.">C3554540</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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||
<div class="spaceAbove">Lymphoproliferative syndrome-2, also known as CD27 deficiency, is an autosomal recessive immunodeficiency disorder associated with persistent symptomatic EBV viremia, hypogammaglobulinemia, and impairment in specific antibody function resulting from impaired T cell-dependent B-cell responses and T-cell dysfunction (summary by van Montfrans et al., 2012). The phenotype can vary significantly, from asymptomatic borderline-low hypogammaglobulinemia, to a full-blown symptomatic systemic inflammatory response with life-threatening EBV-related complications, including hemophagocytic lymphohistiocytosis, a lymphoproliferative disorder, and malignant lymphoma requiring stem cell transplantation (summary by Salzer et al., 2013). For a discussion of genetic heterogeneity of lymphoproliferative syndrome, see XLP1 (308240).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/767454">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_1642840"><div><strong>Familial hemophagocytic lymphohistiocytosis type 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1642840</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.">C4551514</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Familial Hemophagocytic lymphohistiocytosis (FHL) is a rare primary immunodeficiency characterized by a macrophage activation syndrome with an onset usually occurring within a few months or less common several years after birth.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1642840">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_1770239"><div><strong>X-linked lymphoproliferative disease due to SH2D1A deficiency</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1770239</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.">C5399825</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">X-linked lymphoproliferative disease (XLP) in general is characterized by an inappropriate immune response to Epstein-Barr virus (EBV) infection leading to hemophagocytic lymphohistiocytosis (HLH) or severe mononucleosis, dysgammaglobulinemia, and lymphoproliferative disease (malignant lymphoma). The condition primarily affects males. XLP has two recognizable subtypes, XLP1 (due to pathogenic variants in SH2D1A) and XLP2 (due to pathogenic variants in XIAP). HLH / fulminant infectious mononucleosis is the most common presentation regardless of subtype. HLH is characterized as an acute illness with prolonged and high fever, bi- or trilineage cytopenias, and hepatosplenomegaly, which is often severe or fatal. Death is generally secondary to liver failure or multisystem organ dysfunction. In those with XLP1, dys- or hypogammaglobulinemia can lead to varying degrees of humoral immune dysfunction associated with bronchiectasis and recurrent respiratory infections that, if untreated, may result in death. Lymphoproliferative disease (malignant lymphoma) and other lymphoproliferative diseases are specific to XLP1 and often develop in childhood, usually following EBV exposure. Rarer findings in those with XLP1 can include aplastic anemia, vasculitis, and lymphoid granulomatosis. Males with XLP2 are more likely to have HLH without EBV infection, recurrent episodes of HLH (which is not typically seen in those with XLP1), splenomegaly, and gastrointestinal disease, including enterocolitis and perirectal abscesses or fistulae. Rarely, individuals with XLP2 and inflammatory bowel disease have been reported to develop inflammatory liver disease, which can progress to fatal liver failure. Transient hypogammaglobulinemia has been rarely observed in those with XLP2. To date, neither lymphoproliferative disease nor common variable immunodeficiency has been reported in males with XLP2. Heterozygous females rarely have symptoms. There are, however, increasing numbers of reports of affected females with unfavorable (skewed) X-chromosome inactivation favoring the X chromosome with the pathogenic variant who develop HLH, inflammatory bowel disease, and erythema nodosum.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1770239">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_1735911"><div><strong>Immunodeficiency 69</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1735911</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.">C5436498</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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||
<div class="spaceAbove">Immunodeficiency-69 (IMD69) is an autosomal recessive disorder characterized by increased susceptibility to disseminated mycobacterial infection, including after BCG (bacille Calmette-Guerin) vaccination. Affected individuals develop fever, hepatosplenomegaly, leukocytosis, and thrombocytosis during the acute infection. There appears to be normal immunologic function against other pathogens, including viruses and bacteria. Immunologic work-up shows normal parameters, but patient T and NK cells fail to produce gamma-interferon (IFNG) when stimulated in vitro (summary by Kerner et al., 2020). IMD69 is a form of mendelian susceptibility to mycobacterial disease (MSMD) (see, e.g., IMD27A; 209950).</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/1735911">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_1794283"><div><strong>Immunodeficiency 91 and hyperinflammation</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1794283</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.">C5562073</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Immunodeficiency-91 and hyperinflammation (IMD91) is an autosomal recessive complex immunologic disorder characterized by both immunodeficiency and recurrent infections, often to viruses or mycobacteria, as well as by hyperinflammation with systemic involvement. Affected individuals present in infancy with variable features, including fever, infection, thrombocytopenia, renal or hepatic dysfunction, recurrent infections, or seizures. Most patients eventually develop hepatic or renal failure, compromised neurologic function, lymphadenopathy or hepatosplenomegaly, and multiorgan failure resulting in death. More variable features may include intermittent monocytosis, features of hemophagocytic lymphohistiocytosis (HLH), and serologic evidence of hyperinflammation. The disorder is thought to result from dysregulation of the interferon response to viral stimulation in the innate immune system (summary by Le Voyer et al., 2021; Vavassori et al., 2021).</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/1794283">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_1805285"><div><strong>Immunodeficiency 98 with autoinflammation, X-linked</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1805285</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.">C5676883</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">X-linked immunodeficiency-98 with autoinflammation (IMD98) is characterized by onset of recurrent infections associated with lymphoproliferation and autoinflammation in the first decade of life. Mostly males are affected; carrier females may have mild symptoms. Laboratory studies show evidence of immune dysregulation, including hypogammaglobulinemia with reduced memory B cells, skewed T-cell subsets, increased levels of proinflammatory cytokines, activated T cells and monocytes, and autoimmune cytopenias, including neutropenia (Aluri et al., 2021; Fejtkova et al., 2022).</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/1805285">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_1802936"><div><strong>Immunodeficiency 97 with autoinflammation</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1802936</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.">C5676946</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Immunodeficiency-97 with autoinflammation (IMD97) is an autosomal recessive complex immunologic disorder with variable features. Affected individuals present in the first decade of life with inflammatory interstitial lung disease or colitis due to abnormal tissue infiltration by activated T cells. Patients develop autoimmune cytopenias and may have lymphadenopathy; 1 reported patient had features of hemophagocytic lymphohistiocytosis (HLH; see FHL1, 267700). Some patients may have recurrent infections associated with mild lymphopenia, hypogammaglobulinemia, and NK cell dysfunction. Immunologic workup indicates signs of significant immune dysregulation with elevation of inflammatory serum markers, variable immune cell defects involving neutrophils, NK cells, and myeloid cells, and disrupted levels of T regulatory cells (Tregs). Two unrelated patients have been reported (summary by Takeda et al., 2019 and Thian et al., 2020).</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/1802936">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_1803642"><div><strong>Autoinflammatory-pancytopenia syndrome due to DNASE2 deficiency</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1803642</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.">C5676977</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Autoinflammatory-pancytopenia syndrome (AIPCS) is an autosomal recessive disorder characterized by severe anemia and thrombocytopenia apparent from early infancy, hepatosplenomegaly, and recurrent fevers associated with a hyperinflammatory state. Additional systemic features may include chronic diarrhea, proteinuria with renal disease, liver fibrosis with elevated liver enzymes, deforming arthropathy, and vasculitic skin lesions. Some patients may have motor delay or learning difficulties associated with subcortical white matter lesions on brain imaging. Laboratory studies show increased levels of proinflammatory cytokines and increased expression of interferon-stimulated genes (ISGs), consistent with a type I interferonopathy (Rodero et al., 2017). Treatment with a JAK (see 147795) inhibitor (baricitinib) may be effective (Hong et al., 2020).</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/1803642">Condition Record</a></div></div>
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<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1803642" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Autoinflammatory-pancytopenia syndrome due to DNASE2 deficiency</a></div>
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<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_3347" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Chédiak-Higashi syndrome</a></div>
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<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_400366" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Familial hemophagocytic lymphohistiocytosis 2</a></div>
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<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_332383" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Familial hemophagocytic lymphohistiocytosis 3</a></div>
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<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_350245" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Familial hemophagocytic lymphohistiocytosis 4</a></div><div class="jig-moreless" data-jigconfig="class: 'moveDown', moreText: 'See full list (19)', lessText: 'Show less', nodeBefore: 0"><span id="clinMore">
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<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_416514" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Familial hemophagocytic lymphohistiocytosis 5</a></div>
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<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1642840" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Familial hemophagocytic lymphohistiocytosis type 1</a></div>
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<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_357030" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Griscelli syndrome type 2</a></div>
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<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1735911" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Immunodeficiency 69</a></div>
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<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1794283" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Immunodeficiency 91 and hyperinflammation</a></div>
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<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1802936" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Immunodeficiency 97 with autoinflammation</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1805285" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Immunodeficiency 98 with autoinflammation, X-linked</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_765548" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Lymphoproliferative syndrome 1</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_767454" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Lymphoproliferative syndrome 2</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_75704" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Lysinuric protein intolerance</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_7929" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Primary myelofibrosis</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_99306" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Subcutaneous panniculitis-like T-cell lymphoma</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1770239" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">X-linked lymphoproliferative disease due to SH2D1A deficiency</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_336848" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">X-linked lymphoproliferative disease due to XIAP deficiency</a></div></span></div></div>
|
||
</div>
|
||
|
||
<div class="portlet mgSection" id="ID_105">
|
||
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Professional_guidelines">Professional guidelines</h1><a sid="105" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln"><h3 class="subhead">PubMed<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpPM"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/38736876">Heterogeneity of macrophage activation syndrome and treatment progression.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Dong Y,
|
||
Wang T,
|
||
Wu H</span><br />
|
||
<span class="medgenPMjournal">Front Immunol</span>
|
||
2024;15:1389710.
|
||
Epub 2024 Apr 26
|
||
doi: 10.3389/fimmu.2024.1389710.
|
||
<span class="bold">PMID: </span><a href="/pubmed/38736876" target="_blank">38736876</a><a href="/pmc/articles/PMC11082376" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/28477737">Similar but not the same: Differential diagnosis of HLH and sepsis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Machowicz R,
|
||
Janka G,
|
||
Wiktor-Jedrzejczak W</span><br />
|
||
<span class="medgenPMjournal">Crit Rev Oncol Hematol</span>
|
||
2017 Jun;114:1-12.
|
||
Epub 2017 Mar 23
|
||
doi: 10.1016/j.critrevonc.2017.03.023.
|
||
<span class="bold">PMID: </span><a href="/pubmed/28477737" target="_blank">28477737</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/16937360">HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Henter JI,
|
||
Horne A,
|
||
Aricó M,
|
||
Egeler RM,
|
||
Filipovich AH,
|
||
Imashuku S,
|
||
Ladisch S,
|
||
McClain K,
|
||
Webb D,
|
||
Winiarski J,
|
||
Janka G</span><br />
|
||
<span class="medgenPMjournal">Pediatr Blood Cancer</span>
|
||
2007 Feb;48(2):124-31.
|
||
doi: 10.1002/pbc.21039.
|
||
<span class="bold">PMID: </span><a href="/pubmed/16937360" target="_blank">16937360</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22hemophagocytosis%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">See all (34)</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/31151946">Hemophagocytic Lymphohistiocytosis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Skinner J,
|
||
Yankey B,
|
||
Shelton BK</span><br />
|
||
<span class="medgenPMjournal">AACN Adv Crit Care</span>
|
||
2019 Summer;30(2):151-164.
|
||
doi: 10.4037/aacnacc2019463.
|
||
<span class="bold">PMID: </span><a href="/pubmed/31151946" target="_blank">31151946</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/28541437">Hyperferritinemia and inflammation.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Kernan KF,
|
||
Carcillo JA</span><br />
|
||
<span class="medgenPMjournal">Int Immunol</span>
|
||
2017 Nov 1;29(9):401-409.
|
||
doi: 10.1093/intimm/dxx031.
|
||
<span class="bold">PMID: </span><a href="/pubmed/28541437" target="_blank">28541437</a><a href="/pmc/articles/PMC5890889" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/28477737">Similar but not the same: Differential diagnosis of HLH and sepsis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Machowicz R,
|
||
Janka G,
|
||
Wiktor-Jedrzejczak W</span><br />
|
||
<span class="medgenPMjournal">Crit Rev Oncol Hematol</span>
|
||
2017 Jun;114:1-12.
|
||
Epub 2017 Mar 23
|
||
doi: 10.1016/j.critrevonc.2017.03.023.
|
||
<span class="bold">PMID: </span><a href="/pubmed/28477737" target="_blank">28477737</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/25706097">The immunology of Epstein-Barr virus-induced disease.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Taylor GS,
|
||
Long HM,
|
||
Brooks JM,
|
||
Rickinson AB,
|
||
Hislop AD</span><br />
|
||
<span class="medgenPMjournal">Annu Rev Immunol</span>
|
||
2015;33:787-821.
|
||
Epub 2015 Feb 11
|
||
doi: 10.1146/annurev-immunol-032414-112326.
|
||
<span class="bold">PMID: </span><a href="/pubmed/25706097" target="_blank">25706097</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/24782338">Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Fardet L,
|
||
Galicier L,
|
||
Lambotte O,
|
||
Marzac C,
|
||
Aumont C,
|
||
Chahwan D,
|
||
Coppo P,
|
||
Hejblum G</span><br />
|
||
<span class="medgenPMjournal">Arthritis Rheumatol</span>
|
||
2014 Sep;66(9):2613-20.
|
||
doi: 10.1002/art.38690.
|
||
<span class="bold">PMID: </span><a href="/pubmed/24782338" target="_blank">24782338</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Hemophagocytosis%22%20AND%20Etiology%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (250)</a></div><h3 class="subhead">Diagnosis</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/38736876">Heterogeneity of macrophage activation syndrome and treatment progression.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Dong Y,
|
||
Wang T,
|
||
Wu H</span><br />
|
||
<span class="medgenPMjournal">Front Immunol</span>
|
||
2024;15:1389710.
|
||
Epub 2024 Apr 26
|
||
doi: 10.3389/fimmu.2024.1389710.
|
||
<span class="bold">PMID: </span><a href="/pubmed/38736876" target="_blank">38736876</a><a href="/pmc/articles/PMC11082376" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/31151946">Hemophagocytic Lymphohistiocytosis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Skinner J,
|
||
Yankey B,
|
||
Shelton BK</span><br />
|
||
<span class="medgenPMjournal">AACN Adv Crit Care</span>
|
||
2019 Summer;30(2):151-164.
|
||
doi: 10.4037/aacnacc2019463.
|
||
<span class="bold">PMID: </span><a href="/pubmed/31151946" target="_blank">31151946</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/29555368">Approach to pancytopenia: Diagnostic algorithm for clinical hematologists.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Gnanaraj J,
|
||
Parnes A,
|
||
Francis CW,
|
||
Go RS,
|
||
Takemoto CM,
|
||
Hashmi SK</span><br />
|
||
<span class="medgenPMjournal">Blood Rev</span>
|
||
2018 Sep;32(5):361-367.
|
||
Epub 2018 Mar 5
|
||
doi: 10.1016/j.blre.2018.03.001.
|
||
<span class="bold">PMID: </span><a href="/pubmed/29555368" target="_blank">29555368</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/16937360">HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Henter JI,
|
||
Horne A,
|
||
Aricó M,
|
||
Egeler RM,
|
||
Filipovich AH,
|
||
Imashuku S,
|
||
Ladisch S,
|
||
McClain K,
|
||
Webb D,
|
||
Winiarski J,
|
||
Janka G</span><br />
|
||
<span class="medgenPMjournal">Pediatr Blood Cancer</span>
|
||
2007 Feb;48(2):124-31.
|
||
doi: 10.1002/pbc.21039.
|
||
<span class="bold">PMID: </span><a href="/pubmed/16937360" target="_blank">16937360</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/16844525">Griscelli syndrome.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Malhotra AK,
|
||
Bhaskar G,
|
||
Nanda M,
|
||
Kabra M,
|
||
Singh MK,
|
||
Ramam M</span><br />
|
||
<span class="medgenPMjournal">J Am Acad Dermatol</span>
|
||
2006 Aug;55(2):337-40.
|
||
doi: 10.1016/j.jaad.2005.11.1056.
|
||
<span class="bold">PMID: </span><a href="/pubmed/16844525" target="_blank">16844525</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Hemophagocytosis%22%20AND%20Diagnosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (719)</a></div><h3 class="subhead">Therapy</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/38663334">Hemophagocytic lymphohistiocytosis associated with brucellosis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Guo M,
|
||
Zhang L,
|
||
Chen K,
|
||
Huang D,
|
||
Feng C,
|
||
Liu H,
|
||
Quan N,
|
||
Meng F,
|
||
Deng X,
|
||
Liu F</span><br />
|
||
<span class="medgenPMjournal">Diagn Microbiol Infect Dis</span>
|
||
2024 Jul;109(3):116289.
|
||
Epub 2024 Mar 28
|
||
doi: 10.1016/j.diagmicrobio.2024.116289.
|
||
<span class="bold">PMID: </span><a href="/pubmed/38663334" target="_blank">38663334</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/34525183">Characterization of HLH-like manifestations as a CRS variant in patients receiving CD22 CAR T cells.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Lichtenstein DA,
|
||
Schischlik F,
|
||
Shao L,
|
||
Steinberg SM,
|
||
Yates B,
|
||
Wang HW,
|
||
Wang Y,
|
||
Inglefield J,
|
||
Dulau-Florea A,
|
||
Ceppi F,
|
||
Hermida LC,
|
||
Stringaris K,
|
||
Dunham K,
|
||
Homan P,
|
||
Jailwala P,
|
||
Mirazee J,
|
||
Robinson W,
|
||
Chisholm KM,
|
||
Yuan C,
|
||
Stetler-Stevenson M,
|
||
Ombrello AK,
|
||
Jin J,
|
||
Fry TJ,
|
||
Taylor N,
|
||
Highfill SL,
|
||
Jin P,
|
||
Gardner RA,
|
||
Shalabi H,
|
||
Ruppin E,
|
||
Stroncek DF,
|
||
Shah NN</span><br />
|
||
<span class="medgenPMjournal">Blood</span>
|
||
2021 Dec 16;138(24):2469-2484.
|
||
doi: 10.1182/blood.2021011898.
|
||
<span class="bold">PMID: </span><a href="/pubmed/34525183" target="_blank">34525183</a><a href="/pmc/articles/PMC8832442" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/25022357">Hyperinflammation, rather than hemophagocytosis, is the common link between macrophage activation syndrome and hemophagocytic lymphohistiocytosis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Weaver LK,
|
||
Behrens EM</span><br />
|
||
<span class="medgenPMjournal">Curr Opin Rheumatol</span>
|
||
2014 Sep;26(5):562-9.
|
||
doi: 10.1097/BOR.0000000000000093.
|
||
<span class="bold">PMID: </span><a href="/pubmed/25022357" target="_blank">25022357</a><a href="/pmc/articles/PMC4227502" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/18810357">Reactive hemophagocytic syndrome.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Rajam L,
|
||
Prasad V,
|
||
Yatheesha BL</span><br />
|
||
<span class="medgenPMjournal">Indian J Pediatr</span>
|
||
2008 Dec;75(12):1261-3.
|
||
Epub 2008 Sep 22
|
||
doi: 10.1007/s12098-008-0170-y.
|
||
<span class="bold">PMID: </span><a href="/pubmed/18810357" target="_blank">18810357</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/12886898">Hemophagocytic syndrome after Kawasaki disease.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Palazzi DL,
|
||
McClain KL,
|
||
Kaplan SL</span><br />
|
||
<span class="medgenPMjournal">Pediatr Infect Dis J</span>
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<div class="nl"><a target="_blank" href="/pubmed/16844525">Griscelli syndrome.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Malhotra AK,
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<div class="nl"><a target="_blank" href="/pubmed/12886898">Hemophagocytic syndrome after Kawasaki disease.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Palazzi DL,
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McClain KL,
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Kaplan SL</span><br />
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<span class="medgenPMjournal">Pediatr Infect Dis J</span>
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2003 Jul;22(7):663-6.
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<span class="bold">PMID: </span><a href="/pubmed/12886898" target="_blank">12886898</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/11076718">Hemophagocytic syndromes and infection.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Fisman DN</span><br />
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2000 Nov-Dec;6(6):601-8.
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doi: 10.3201/eid0606.000608.
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|
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<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Hemophagocytosis%22%20AND%20Prognosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (400)</a></div><h3 class="subhead">Clinical prediction guides</h3>
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<div class="nl"><a target="_blank" href="/pubmed/37495396">Human TLR8 induces inflammatory bone marrow erythromyeloblastic islands and anemia in SLE-prone mice.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Maria NI,
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Papoin J,
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Raparia C,
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Sun Z,
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Josselsohn R,
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Lu A,
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Katerji H,
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Syeda MM,
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Polsky D,
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Paulson R,
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Kalfa T,
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2023 Oct;6(10)
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Epub 2023 Jul 26
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doi: 10.26508/lsa.202302241.
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<span class="bold">PMID: </span><a href="/pubmed/37495396" target="_blank">37495396</a><a href="/pmc/articles/PMC10372407" target="_blank" class="PubMedFree">Free PMC Article</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/31335286">Parvovirus B19 Myocarditis: Looking Beyond the Heart.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Izquierdo-Blasco J,
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Salcedo Allende MT,
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Codina Grau MG,
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Gran F,
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Balcells J</span><br />
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<span class="medgenPMjournal">Pediatr Dev Pathol</span>
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2020 Mar-Apr;23(2):158-162.
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Epub 2019 Jul 23
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doi: 10.1177/1093526619865641.
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<span class="bold">PMID: </span><a href="/pubmed/31335286" target="_blank">31335286</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/28541437">Hyperferritinemia and inflammation.</a></div>
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||
<div class="portlet_content ln"><span class="medgenPMauthor">Kernan KF,
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Carcillo JA</span><br />
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<span class="medgenPMjournal">Int Immunol</span>
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2017 Nov 1;29(9):401-409.
|
||
doi: 10.1093/intimm/dxx031.
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<span class="bold">PMID: </span><a href="/pubmed/28541437" target="_blank">28541437</a><a href="/pmc/articles/PMC5890889" target="_blank" class="PubMedFree">Free PMC Article</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/24782338">Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome.</a></div>
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Lambotte O,
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<span class="bold">PMID: </span><a href="/pubmed/24782338" target="_blank">24782338</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/17627615">Pathogenesis and mechanism of disease progression from hemophagocytic lymphohistiocytosis to Epstein-Barr virus-associated T-cell lymphoma: nuclear factor-kappa B pathway as a potential therapeutic target.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Chuang HC,
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<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Hemophagocytosis%22%20AND%20Clinical%20prediction%20guides%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (195)</a></div></div>
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|
||
|
||
<div class="portlet mgSection" id="ID_104">
|
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_systematic_reviews">Recent systematic reviews</h1><a sid="104" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
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<div class="portlet_content ln">
|
||
<div class="nl"><a target="_blank" href="/pubmed/33481385">Postmortem Findings Associated With SARS-CoV-2: Systematic Review and Meta-analysis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Satturwar S,
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||
Fowkes M,
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||
Farver C,
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||
Wilson AM,
|
||
Eccher A,
|
||
Girolami I,
|
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Pujadas E,
|
||
Bryce C,
|
||
Salem F,
|
||
El Jamal SM,
|
||
Paniz-Mondolfi A,
|
||
Petersen B,
|
||
Gordon RE,
|
||
Reidy J,
|
||
Fraggetta F,
|
||
Marshall DA,
|
||
Pantanowitz L</span><br />
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||
<span class="medgenPMjournal">Am J Surg Pathol</span>
|
||
2021 May 1;45(5):587-603.
|
||
doi: 10.1097/PAS.0000000000001650.
|
||
<span class="bold">PMID: </span><a href="/pubmed/33481385" target="_blank">33481385</a><a href="/pmc/articles/PMC8132567" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/32679608">Clinical, immunological and genetic findings in patients with UNC13D deficiency (FHL3): A systematic review.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Amirifar P,
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||
Ranjouri MR,
|
||
Abolhassani H,
|
||
Moeini Shad T,
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||
Almasi-Hashiani A,
|
||
Azizi G,
|
||
Moamer S,
|
||
Aghamohammadi A,
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||
Yazdani R</span><br />
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<span class="medgenPMjournal">Pediatr Allergy Immunol</span>
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||
2021 Jan;32(1):186-197.
|
||
Epub 2020 Aug 24
|
||
doi: 10.1111/pai.13323.
|
||
<span class="bold">PMID: </span><a href="/pubmed/32679608" target="_blank">32679608</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/32750378">A review of the main histopathological findings in coronavirus disease 2019.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Vasquez-Bonilla WO,
|
||
Orozco R,
|
||
Argueta V,
|
||
Sierra M,
|
||
Zambrano LI,
|
||
Muñoz-Lara F,
|
||
López-Molina DS,
|
||
Arteaga-Livias K,
|
||
Grimes Z,
|
||
Bryce C,
|
||
Paniz-Mondolfi A,
|
||
Rodríguez-Morales AJ</span><br />
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||
<span class="medgenPMjournal">Hum Pathol</span>
|
||
2020 Nov;105:74-83.
|
||
Epub 2020 Aug 2
|
||
doi: 10.1016/j.humpath.2020.07.023.
|
||
<span class="bold">PMID: </span><a href="/pubmed/32750378" target="_blank">32750378</a><a href="/pmc/articles/PMC7395947" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/23104659">Haemophagocytic syndrome in rheumatic patients. A systematic review.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Atteritano M,
|
||
David A,
|
||
Bagnato G,
|
||
Beninati C,
|
||
Frisina A,
|
||
Iaria C,
|
||
Bagnato G,
|
||
Cascio A</span><br />
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<span class="medgenPMjournal">Eur Rev Med Pharmacol Sci</span>
|
||
2012 Oct;16(10):1414-24.
|
||
<span class="bold">PMID: </span><a href="/pubmed/23104659" target="_blank">23104659</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/23104648">Secondary hemophagocytic lymphohistiocytosis in zoonoses. A systematic review.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Cascio A,
|
||
Pernice LM,
|
||
Barberi G,
|
||
Delfino D,
|
||
Biondo C,
|
||
Beninati C,
|
||
Mancuso G,
|
||
Rodriguez-Morales AJ,
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||
Iaria C</span><br />
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<span class="medgenPMjournal">Eur Rev Med Pharmacol Sci</span>
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||
2012 Oct;16(10):1324-37.
|
||
<span class="bold">PMID: </span><a href="/pubmed/23104648" target="_blank">23104648</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Hemophagocytosis%22%20AND%20systematic%5Bsb%5D%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (7)</a></div></div>
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<h2 class="offscreen_noflow">Supplemental Content</h2>
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<a href="/pubmed?term=Hemophagocytosis%20AND%20humans[mesh]%20AND%20review[publication%20type]" ref="ncbi_uid=&discoId=gtr_reviews&linkpos=2&linkpostotal=2" target="_blank">Reviews in PubMed</a>
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