auto-immuun lymfoproliferatief syndroom
auto-immuunziekte
autosomaal dominante hereditaire aandoening
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auto-immuun lymfoproliferatief syndroom door cytotoxische T-lymfocyt-geassocieerde eiwit 4 haplo-insufficiëntie (aandoening)
auto-immuun lymfoproliferatief syndroom door cytotoxische T-lymfocyt-geassocieerde eiwit 4 haplo-insufficiëntie
ALPS door CTLA-4-haplo-insufficiëntie
Autoimmune lymphoproliferative syndrome due to CTLA4 haploinsufficiency
Autoimmune lymphoproliferative syndrome due to cytotoxic T-lymphocyte associated protein 4 haploinsufficiency
CTLA-4 haploinsufficiency with autoimmune infiltration disease
A rare primary immunodeficiency characterized by variable combination of enteropathy, hypogammaglobulinemia, recurrent respiratory infections, granulomatous lymphocytic interstitial lung disease, lymphocytic infiltration of non-lymphoid organs (intestine, lung, brain, bone marrow, kidney), autoimmune thrombocytopenia or neutropenia, autoimmune hemolytic anemia and lymphadenopathy.
Id1197361002
StatusPrimitive
Associated morphologylymfoproliferatieve aandoening
Finding sitelymfocyt
Pathological processauto-immuunproces
SNOMED CT to Orphanet simple map436159
SNOMED CT to ICD-10 extended map
TargetD84.8
RuleTRUE
AdviceALWAYS D84.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified