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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 (ras homolog family member H) deficiency
T-cell immunodeficiency due to RHOH deficiency
T-cell immunodeficiency due to ras homolog family member H deficiency
A rare primary immunodeficiency with characteristics of 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.
Id770785002
StatusPrimitive
Pathological processafwijkend immuunproces
referentieset met complexe 'mapping' naar ICD-10
TargetD84.8
RuleTRUE
AdviceALWAYS D84.8
CorrelationSNOMED CT source code to target map code correlation not specified