ORPHA:158038
Primary hemophagocytic lymphohistiocytosis
Synonyms: Genetic HLH · Genetic hemophagocytic lymphohistiocytosis · Primary HLH
- Type
- Clinical group
- Group
- Group of disorders
- Status
- active
This term does not characterize a disease but a group of diseases. To learn about the diseases included under this term, you can consult the classifications.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary hemophagocytic lymphohistiocytosis | Genetic HLH · Genetic hemophagocytic lymphohistiocytosis · Primary HLH |
| fr | Lymphohistiocytose hémophagocytaire primaire | HLH génétique · HLH primaire · Hémophagocytose lymphohistiocytaire génétique · Hémophagocytose lymphohistiocytaire primaire · Lymphohistiocytose hémophagocytaire génétique |
| de | Lymphohistiozytose, hämophagozytische, primäre | Lymphohistiozytose, hämophagozytische, genetisch bedingte · Primäre HLH |
| es | Linfohistiocitosis hemofagocítica primaria | LHH genética · LHH primaria · Linfohistiocitosis hemofagocítica genética |
| it | Linfoistiocitosi emofagocitica primaria | HLH gentica · HLH primaria · Linfoistiocitosi emofagocitica genetica |
| nl | Primaire hemofagocytaire lymfohistiocytose | Genetische HLH · Genetische hemofagocytaire lymfohistiocytose · Primaire HLH |
| pt | Linfo-histiocitose hemofagocítica primária | HLH primária · Linfo-histiocitose hemofagocítica genética |
| pl | Pierwotna limfohistiocytoza hemofagocytarna | Genetyczna limfohistiocytoza hemofagocytarna |
| cs | Primární hemofagocytující lymfohistiocytóza | Geneticky podmíněná hemofagocytující lymfohistiocytóza |
| uk | Первинний гемофагоцитарний лімфогістіоцитоз | Генетичний гемофагоцитарний лімфогістіоцитоз · Генетичний HLH · Первинний HLH |
| zh | 原发性噬血细胞性淋巴组织细胞增多症 | 遗传性噬血细胞性淋巴组织细胞增多症 |
| tr | Primer hemofagositik lenfohistiyositoz | Genetik hemofagositik lenfohistiyositoz |
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-11 | 4A01.23 | E | specific | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (3):
- ORPHA:158032Hemophagocytic syndrome
rare immunological diseases - ORPHA:169361Immune dysregulation disease with immunodeficiency
rare genetic diseases; rare immunological diseases - ORPHA:506219Rare disorder potentially indicated for hematopoietic stem cell transplant
rare transplant-related disorders
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
0 recordsNo prevalence record in this release.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/158038
- This record as JSON: /data/d/158038.json