raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:179006

Primary immunodeficiency due to a defect in adaptive immunity

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

12 of 12 languages
LangNameSynonyms
enPrimary immunodeficiency due to a defect in adaptive immunity
frDéficit immunitaire primaire par défaut de l'immunité adaptativeImmunodéficience primaire par défaut de l'immunité adaptative
dePrimärer Immundefekt durch Störung der adaptiven Immunität
esInmunodeficiencia primaria por defecto en la inmunidad adaptativa
itImmunodeficienza primitiva da difetto dell'immunità adattativa
nlPrimaire immuundeficiëntie door defect van verworven immuniteit
ptDeficiência na imunidade adaptativa
plPierwotny Niedobór odporności z powodu defektu odporności nabytej
csPrimární imunodeficience způsobená poruchou specifické imunity
ukПервинний імунодефіцит внаслідок дефекту адаптивного імунітету
zh适应性免疫缺陷所致原发性免疫缺陷
trAdaptif bağışıklıktaki bir defekte bağlı primer immün yetmezlik
2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-114A01Especificvalidated
MONDO15823Evalidated
UMLSC5680501Evalidated

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 (5):

Codes that Orphanet moved or referred to this one:

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