autosomaal recessieve hereditaire aandoening	primaire immunodeficiëntie
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T-cel-immuundeficiëntie met epidermodysplasia verruciformis (aandoening)
	T-cel-immuundeficiëntie met epidermodysplasia verruciformis
	T-cel-immunodeficiëntie door RHOH-deficiëntie
	T-cell immunodeficiency with epidermodysplasia verruciformis
	T-cell immunodeficiency due to RHOH deficiency T-cell immunodeficiency due to ras homolog family member H deficiency T-cell immunodeficiency due to RHOH (ras homolog family member H) deficiency
	A rare primary immunodeficiency characterized by increased susceptibility to infection by human papillomavirus, presenting in childhood with disseminated flat wart-like cutaneous lesions. Burkitt lymphoma has also been reported. Whilst total T-cell counts are normal, there is impaired TCR signaling, profound peripheral naive T-cell lymphopenia with memory T-cells displaying an exhaustion phenotype.

Id	770785002
Status	Primitive

Pathological process

afwijkend immuunproces

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	D84.8
Term	Overige gespecificeerde immunodeficiënties

SNOMED CT to Orphanet simple map

324294

SNOMED CT to ICD-10 extended map

Target	D84.8
Rule	TRUE
Advice	ALWAYS D84.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified