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T-cell immunodeficiency with epidermodysplasia verruciformis

MONDO:0017925

T-cell immunodeficiency with epidermodysplasia verruciformis is 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.

Also known as: T-cell immunodeficiency due to RHOH deficiency

41 clinical trials for this condition and its sub-types, 0 tagged with T-cell immunodeficiency with epidermodysplasia verruciformis itself.

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