ORPHA:39812

Graft versus host disease

Synonyms: GvHD

Type: Disease
Group: Disorder
Inheritance: Not applicable
Age of onset: All ages
Status: active

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.

Names in Orphadata languages

12 of 12 languages

Lang	Name	Synonyms
en	Graft versus host disease	GvHD
fr	Maladie du greffon contre l'hôte
de	Graft versus host-Krankheit	GVHD
es	Enfermedad de injerto contra hospedador	EICH · Enfermedad de injerto versus huésped
it	Malattia da rigetto del trapianto	GVH
nl	Graft-versus-hostziekte	GvHD · GvHZ · Omgekeerde afstotingsziekte · Transplantaat-tegen-gastheerziekte
pt	Doença do enxerto versus hospedeiro	GVH
pl	Choroba przeszczep przeciwko gospodarzowi	GVH
cs	Reakce štěpu proti hostiteli	GVHD
uk	Хвороба трансплантата проти хазяїна	= en
zh	移植物抗宿主病	GVH
tr	Graft versus host hastalığı	GVH

Definition in:

Cross-references

qualifier = Orphanet mapping relation

Source	Code	Qualifier	ICD relation	Validation
OMIM	614395	E		validated
ICD-10	T86.0	NTBT	inclusion	validated
ICD-11	4B24	E	specific	validated
MONDO	0013730	E		validated
MeSH	D006086	E		validated
UMLS	C0018133	E		validated
MedDRA	10018651	E		validated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

Classification

Preferential parent (Orphanet linearisation):

ORPHA:98004Rare immune disease

Parents in the classifications (2):

ORPHA:565779Rare disorder potentially indicated for transplant or complication after transplantation
rare transplant-related disorders
ORPHA:98004Rare immune disease
rare immunological diseases

Children (2):

Genes

0 direct

Orphanet records no gene directly on this code.

Prevalence and incidence

1 record

Type	Class	Value	Area	Qualification	Validation	Source
Point prevalence	1-9 / 100 000	5 / 100 000	Europe	Value and class	Validated	European Medicines Agency 2018[OTHER]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

HPO phenotypes

41 annotations · validated 2019-05-22

HPO	Term (as annotated by Orphanet)	Diagnostic
Very frequent (99-80%)
HP:0011123	Inflammatory abnormality of the skin
Frequent (79-30%)
HP:0200123	Chronic hepatitis
HP:0002014	Diarrhea
HP:0002910	Elevated circulating hepatic transaminase concentration
HP:0040186	Maculopapular exanthema
HP:0000155	Oral ulcer
HP:0002719	Recurrent infections
HP:0200041	Skin erosion
HP:0010280	Stomatitis
Occasional (29-5%)
HP:0002027	Abdominal pain
HP:0200119	Acute hepatitis
HP:0001369	Arthritis
HP:0031359	Cutaneous sclerotic plaque
HP:0003155	Elevated circulating alkaline phosphatase concentration
HP:0100537	Fasciitis
HP:0004386	Gastrointestinal inflammation
HP:0012156	Hemophagocytosis
HP:0001433	Hepatosplenomegaly
HP:0002904	Hyperbilirubinemia
HP:0100533	Inflammatory abnormality of the eye
HP:0000737	Irritability
HP:0000952	Jaundice
HP:0031452	Lichenoid skin lesion
HP:0002996	Limited elbow movement
HP:0006467	Limited shoulder movement
HP:0100614	Myositis
HP:0002018	Nausea
HP:0002090	Pneumonia
HP:0002113	Pulmonary infiltrates
HP:0031123	Recurrent gastroenteritis
HP:0040189	Scaling skin
HP:0003202	Skeletal muscle atrophy
HP:0001649	Tachycardia
HP:0002013	Vomiting
Very rare (<4-1%)
HP:0000633	Decreased lacrimation
HP:0005679	Dupuytren contracture
HP:0001508	Failure to thrive
HP:0009125	Lipodystrophy
HP:0002716	Lymphadenopathy
HP:0005198	Stiff interphalangeal joints
HP:0000211	Trismus

Run the differential on the 1 obligate / very frequent terms

Annotation source: 30855823[PMID]_31042160[PMID]_31058026[PMID]_31075084[PMID]_31089279[PMID]_31089281[PMID]_30106904[PMID]

Sources for this card

Orphanet page: orpha.net/en/disease/detail/39812
This record as JSON: /data/d/39812.json