raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:664482

Primary hemophagocytic lymphohistiocytosis without hypopigmentation

Synonyms: Genetic HLH without hypopigmentation · Genetic hemophagocytic lymphohistiocytosis without hypopigmentation · Primary HLH without hypopigmentation

Type
Category
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.

1

Names in Orphadata languages

10 of 12 languages
LangNameSynonyms
enPrimary hemophagocytic lymphohistiocytosis without hypopigmentationGenetic HLH without hypopigmentation · Genetic hemophagocytic lymphohistiocytosis without hypopigmentation · Primary HLH without hypopigmentation
frLymphohistiocytose hémophagocytaire primaire sans hypopigmentationHLH primaire sans hypopigmentation · Hémophagocytose lymphohistiocytaire génétique sans hypopigmentation · Hémophagocytose lymphohistiocytaire primaire sans hypopigmentation · Lymphohistiocytose hémophagocytaire génétique sans hypopigmentation
dePrimäre hämophagozytische Lymphohistiozytose ohne HypopigmentierungPrimäre HLH ohne Hypopigmentierung · Genetisch bedingte HLH ohne Hypopigmentierung
esLinfohistiocitosis hemofagocítica primaria sin hipopigmentaciónLHH familiar sin hipopigmentación · LHH genética sin hipopigmentación · LHH primaria sin hipopigmentación · Linfohistiocitosis hemofagocítica familiar sin hipopigmentación · Linfohistiocitosis hemofagocítica genética sin hipopigmentación
itLinfoistiocitosi emofagocitica primaria senza ipopigmentazioneHLH genetica senza ipopigmentazione · HLH primitiva senza ipopigmentazione · Linfoistiocitosi emofagocitica genetica senza ipopigmentazione
nlPrimaire hemofagocytaire lymfohistiocytose zonder hypopigmentatieGenetische HLH zonder hypopigmentatie · Genetische hemofagocytaire lymfohistiocytose zonder hypopigmentatie · Primaire HLH zonder hypopigmentatie
ptLinfohistiocitose hemofagocítica primária sem hipopigmentaçãoHLH genética sem hipopigmentação · HLH primária sem hipopigmentação · Linfohistiocitose hemofagocítica genética sem hipopigmentação
plPierwotna limfohistiocytoza hemofagocytarna bez hipopigmentacji= en
csPrimární hemofagocytární lymfohistiocytóza bez hypopigmentace= en
ukПервинний гемофагоцитарний лімфогістіоцитоз без гіпопігментаціїГенетичний гемофагоцитарний лімфогістіоцитоз без гіпопігментації · Генетична HLH без гіпопігментації · Первинна HLH без гіпопігментації
2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
UMLSC5925055Evalidated

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

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

Children (3):

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card