Combined immunodeficiency due to ZAP70 deficiency
ORPHA:911· ICD-10 D81.8
Definition
A very rare, severe, genetic, combined immunodeficiency disorder characterized by lymphocytosis, decreased peripheral CD8+ T-cells, and presence of normal circulating CD4+ T-cells, leading to immune dysfunction.
- Prevalence
- Unknown
- Inheritance
- Autosomal recessive
- Age of onset
- Childhood