ORPHA:158048

Hemophagocytic syndrome associated with an infection

Synonyms: IAHS · VAHS · Virus-associated hemophagocytic syndrome

Orphadata ships no English definition for this code.

Names in Orphadata languages

12 of 12 languages

Lang	Name	Synonyms
en	Hemophagocytic syndrome associated with an infection	IAHS · VAHS · Virus-associated hemophagocytic syndrome
fr	Syndrome hémophagocytaire associé à une infection	Hémophagocytaire, syndrome, viro-induit · IAHS · Syndrome hémophagocytaire viro-induit · VAHS
de	Hämophagozytisches Syndrom, infektionsbedingtes	Hämophagozytisches Syndrom, Virus-assoziiertes · IAHS · VAHS
es	Síndrome hemofagocítico asociado a infección	Síndrome hemofagocítico asociado a virus · SHAI · SHAV
it	Sindrome emofagocitica associata a infezione	IAHS · Sindrome emofagocitica associata a virus · VAHS
nl	Hemofagocytair syndroom geassocieerd met infectie	IAHS · VAHS · Virusgeassocieerde hemofagocytis-syndroom
pt	Síndrome hemofagocítico associado a infecção
pl	Zespół hemofagocytarny towarzyszący infekcji	IAHS · VAHS · Zespół hemofagocytarny towarzyszący infekcji wirusowej
cs	Hemofagocytující syndrom související s infekcí	IAHS · VASH · Sekundární hemofagocytující syndrom
uk	Гемофагоцитарний синдром, повязаний з інфекцією	IAHS · VAHS · Вірус-асоційований гемофагоцитарний синдром
zh	感染相关噬血细胞综合征	病毒相关性噬血细胞综合征
tr	Bir enfeksiyonla ilişkili hemofagositik sendrom	IAHS · VAHS · Virüs-ilişkili hemofagositik sendrom

qualifier = Orphanet mapping relation

Source	Code	Qualifier	ICD relation	Validation
ICD-10	D76.2	E	specific	validated
ICD-11	3B62.5	E	specific	validated
UMLS	C5207031	E		validated
GARD	7857	E		validated

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

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

0 direct

Orphanet records no gene directly on this code.

1 record

Type	Class	Value	Area	Qualification	Validation	Source
Point prevalence	Unknown	—	Worldwide	Class only	Not yet validated	ORPHANET

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

35 annotations · validated 2021-11-24

HPO	Term (as annotated by Orphanet)	Diagnostic
Obligate (100%)
HP:0012156	Hemophagocytosis	Diagnostic criterion
Very frequent (99-80%)
HP:0025540	Abnormal T cell subset distribution	Diagnostic criterion
HP:0031406	Abnormal cytokine signaling
HP:0012647	Abnormal inflammatory response
HP:0001945	Fever	Diagnostic criterion
HP:0001876	Pancytopenia
HP:0032169	Severe infection
HP:0031691	Severe viral infection
HP:0001744	Splenomegaly	Diagnostic criterion
Frequent (79-30%)
HP:0040089	Abnormal natural killer cell count	Diagnostic criterion
HP:0000707	Abnormality of the nervous system
HP:0001903	Anemia	Diagnostic criterion
HP:0032204	Chronic active Epstein-Barr virus infection
HP:0001875	Decreased total neutrophil count	Diagnostic criterion
HP:0005521	Disseminated intravascular coagulation
HP:0002910	Elevated circulating hepatic transaminase concentration
HP:0002240	Hepatomegaly
HP:0002155	Hypertriglyceridemia	Diagnostic criterion
HP:0011900	Hypofibrinogenemia
HP:0003281	Increased circulating ferritin concentration	Diagnostic criterion
HP:0001873	Thrombocytopenia	Diagnostic criterion
Occasional (29-5%)
HP:0001251	Ataxia
HP:0012229	CSF pleocytosis
HP:0006824	Cranial nerve paralysis
HP:0001260	Dysarthria
HP:0002152	Hyperproteinemia
HP:0020101	Invasive fungal infection
HP:0031179	Nuchal rigidity
HP:0032260	Opportunistic bacterial infection
HP:0030891	Periventricular white matter hyperintensities
HP:0001250	Seizure
HP:0031692	Severe cytomegalovirus infection
Very rare (<4-1%)
HP:0001298	Encephalopathy
HP:0032256	Histoplasmosis
HP:0031700	Invasive parasitic infection

Annotation source: 31339233[PMID]_32560584[PMID]