{"orpha":39812,"name":"Graft versus host disease","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["GvHD"],"definition":"A rare immune disease characterized by donor-derived immune cells, primarily T cells, recognizing the recipient's tissues as foreign and mounting an immune response against them. It is a significant complication following allogeneic hematopoietic cell transplantation (alloHCT). Graft versus host disease (GVHD) is classified into acute (aGVHD) and chronic (cGVHD) forms.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-39812/","orphanet_url":"https://www.orpha.net/en/disease/detail/39812","names":{"fr":{"name":"Maladie du greffon contre l'hôte","definition":"Maladie immunitaire rare caractérisée par une réaction des cellules immunocompétentes du donneur, principalement des cellules T, contre les tissus de l'hôte, reconnus comme étrangers. Il s'agit d'une complication importante après une allogreffe de cellules hématopoïétiques (alloHCT). On distingue une forme aiguë (aGVHD) et une forme chronique (cGVHD) de la maladie du greffon contre l'hôte (GVHD)."},"de":{"name":"Graft versus host-Krankheit","synonyms":["GVHD"],"definition":"Eine seltene Immunkrankheit, bei der vom Spender stammende Immunzellen - in erster Linie T-Zellen - das Gewebe des Empfängers als fremd erkennen und eine Immunreaktion dagegen auslösen. Sie stellt eine bedeutende Komplikation nach allogener hämatopoetischer Zelltransplantation (alloHCT) dar. Man unterscheidet zwischen akuter (aGVHD) und chronischer (cGVHD) Graft-versus-Host-Krankheit (GVHD)."},"es":{"name":"Enfermedad de injerto contra hospedador","synonyms":["EICH","Enfermedad de injerto versus huésped"],"definition":"Es una enfermedad inmunológica poco frecuente caracterizada por el reconocimiento por parte de las células inmunológicas del donante, principalmente los linfocitos T, de los tejidos del receptor como extraños y la generación de una respuesta inmunológica contra ellos. Es una complicación importante tras el trasplante alogénico de progenitores hematopoyéticos (alo-TPH). La enfermedad de injerto contra receptor (EICR se clasifica en aguda (EICRa) y crónica (EICRc).Es una enfermedad inmunológica poco frecuente caracterizada por el reconocimiento por parte de las células inmunológicas del donante, principalmente los linfocitos T, de los tejidos del receptor como extraños y la generación de una respuesta inmunológica contra ellos. Es una complicación importante tras el trasplante alogénico de progenitores hematopoyéticos (alo-TPH). La enfermedad de injerto contra receptor (EICR se clasifica en aguda (EICRa) y crónica (EICRc)."},"it":{"name":"Malattia da rigetto del trapianto","synonyms":["GVH"]},"nl":{"name":"Graft-versus-hostziekte","synonyms":["GvHD","GvHZ","Omgekeerde afstotingsziekte","Transplantaat-tegen-gastheerziekte"],"definition":"Een zeldzame immuunziekte, gekenmerkt door immuuncellen afkomstig van de donor, hoofdzakelijk T-cellen, die de weefsels van de ontvanger herkennen als lichaamsvreemd en hiertegen die een immuunrespons veroorzaken. Het is een significante complicatie na allogene hematopoëtische celtransplantatie (alloHCT). Graft-versus-hostziekte (GVHD) of omgekeerde afstotingsziekte wordt onderverdeeld in acute (aGVHD) en chronische (cGVHD) vormen."},"pt":{"name":"Doença do enxerto versus hospedeiro","synonyms":["GVH"]},"pl":{"name":"Choroba przeszczep przeciwko gospodarzowi","synonyms":["GVH"]},"cs":{"name":"Reakce štěpu proti hostiteli","synonyms":["GVHD"]},"uk":{"name":"Хвороба трансплантата проти хазяїна","synonyms":["GvHD"],"definition":"A rare disease that occurs after allogeneic hematopoietic stem cell transplant and is a reaction of donor immune cells against host tissues. Activated donor T cells damage host epithelial cells after an inflammatory cascade that begins with the preparative regimen."},"zh":{"name":"移植物抗宿主病","synonyms":["GVH"]},"tr":{"name":"Graft versus host hastalığı","synonyms":["GVH"]}},"xrefs":[{"source":"OMIM","code":"614395","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"T86.0","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"4B24","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"437372167"},{"source":"MONDO","code":"0013730","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D006086","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0018133","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10018651","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98004,"name":"Rare immune disease"},"parents":[{"orpha":565779,"name":"Rare disorder potentially indicated for transplant or complication after transplantation","classifications":[233]},{"orpha":98004,"name":"Rare immune disease","classifications":[195]}],"children":[{"orpha":99920,"name":"Acute graft versus host disease","type":"Clinical subtype"},{"orpha":99921,"name":"Chronic graft versus host disease","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":5.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2018[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-05-22","source":"30855823[PMID]_31042160[PMID]_31058026[PMID]_31075084[PMID]_31089279[PMID]_31089281[PMID]_30106904[PMID]","terms":[{"hpo":"HP:0011123","label":"Inflammatory abnormality of the skin","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200123","label":"Chronic hepatitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002910","label":"Elevated circulating hepatic transaminase concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0040186","label":"Maculopapular exanthema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000155","label":"Oral ulcer","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002719","label":"Recurrent infections","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200041","label":"Skin erosion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010280","label":"Stomatitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200119","label":"Acute hepatitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001369","label":"Arthritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031359","label":"Cutaneous sclerotic plaque","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003155","label":"Elevated circulating alkaline phosphatase concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100537","label":"Fasciitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004386","label":"Gastrointestinal inflammation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012156","label":"Hemophagocytosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001433","label":"Hepatosplenomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002904","label":"Hyperbilirubinemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100533","label":"Inflammatory abnormality of the eye","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000737","label":"Irritability","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000952","label":"Jaundice","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031452","label":"Lichenoid skin lesion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002996","label":"Limited elbow movement","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006467","label":"Limited shoulder movement","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100614","label":"Myositis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002090","label":"Pneumonia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002113","label":"Pulmonary infiltrates","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031123","label":"Recurrent gastroenteritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0040189","label":"Scaling skin","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003202","label":"Skeletal muscle atrophy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001649","label":"Tachycardia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000633","label":"Decreased lacrimation","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0005679","label":"Dupuytren contracture","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001508","label":"Failure to thrive","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0009125","label":"Lipodystrophy","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0005198","label":"Stiff interphalangeal joints","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000211","label":"Trismus","frequency":"Very rare (<4-1%)","criteria":null}]}}