autosomaal recessieve hereditaire aandoening
gecombineerde immunodeficiëntie
hereditair tumorpredispositiesyndroom
lymfoproliferatieve aandoening
||||
autosomaal recessieve lymfoproliferatieve ziekte (aandoening)
autosomaal recessieve lymfoproliferatieve ziekte
autosomaal recessieve lymfoproliferatieve aandoening
Autosomal recessive lymphoproliferative disease
A rare autosomal recessive primary immunodeficiency characterized by Epstein-Barr virus (EBV)-triggered lymphoproliferative disorders such as malignant B-cell proliferation, Hodgkin lymphoma, B-cell lymphoma and EBV-driven hemophagocytic lymphohistiocytosis (HLH). Aplastic anemia and inflammatory disorders such as uveitis and oral ulcers are also observed.
Id771309000
StatusPrimitive
Associated morphologylymfoproliferatieve aandoening
Pathological processafwijkend immuunproces
DHD Diagnosis thesaurus reference set
SNOMED CT to Orphanet simple map238505
SNOMED CT to ICD-10 extended map
TargetD47.9
RuleTRUE
AdviceALWAYS D47.9 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE
CorrelationSNOMED CT source code to target map code correlation not specified