Hemophagocytic syndrome
Synonyms: HLH · Hemophagocytic lymphohistiocytosis
- Type
- Category
- Group
- Group of disorders
- Age of onset
- Adolescent, Childhood, Infancy
- Status
- active
Hemophagocytic syndrome (HPS) is a rare immune disease and a potentially life-threatening disorder characterized by cytokine storm and overwhelming inflammation causing fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. It can be either primary due to a genetic defect (primary hemophagocytic lymphohistiocytosis ), or secondary to malignancies, to infections, most commonly with viruses such as Epstein-Barr virus or cytomegalovirus, human immunodeficiency virus, or to autoimmune disorders such as systemic lupus erythematosus or adult-onset Still disease (secondary hemophagocytic lymphohistiocytosis).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Hemophagocytic syndrome | HLH · Hemophagocytic lymphohistiocytosis |
| fr | Syndrome hémophagocytaire | HLH · Lymphohistiocytose hémophagocytaire · Lymphohistiocytose hémophagocytique |
| de | Hämophagozytose-Syndrom | HLH · Lymphohistiozytose, hämophagozytische |
| es | Síndrome hemofagocítico | HLH · Linfohistiocitosis hemofagocítica |
| it | Sindrome emofagocitica | HLH · Linfoistiocitosi emofagocitica |
| nl | Hemofagocytair syndroom | HLH · Hemofagocytaire lymfohistiocytose |
| pt | Síndrome hemofagocítico | |
| pl | Zespół hemofagocytarny | HLH · Limfohistiocytoza hemofagocytarna |
| cs | Hemofagocytický syndrom | HLH · Hemofagocytická lymfohistiocytóza |
| uk | Гемофагоцитарний синдром | Гемофагоцитарний лімфогістіоцитоз · HLH |
| zh | 噬血细胞综合征 | 噬血细胞性淋巴组织细胞增多症 |
| tr | Hemofagositik sendrom | HLH · Hemofagositik lenfohistiyositoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-11 | 4A01.23 | NTBT | index | validated |
| MONDO | 0015540 | E | validated | |
| MONDO | 15540 | E | validated | |
| UMLS | C0024291 | E | validated | |
| MedDRA | 10058125 | 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):
Parents in the classifications (1):
- ORPHA:98004Rare immune disease
rare immunological diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
| Annual incidence | 1-9 / 100 000 | 0.198 / 100 000 | United Kingdom | Value and class | Validated | 34875131[PMID] |
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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/158032
- This record as JSON: /data/d/158032.json