NIK deficiency

Engelsk navn: NIK deficiency
Engelske synonym: Primary immunodeficiency with multifaceted aberrant lymphoid immunity


A rare, genetic, primary combined T and B cell immunodeficiency characterized by recurrent, severe viral and bacterial infections. Immunologic findings include decreased immunoglobulin levels, decreased numbers of B and NK cells, reduced relative CD19+ B cells in peripheral blood, impaired memory responses to viral infections and defective antigen-specific T-cell proliferation.

Fra Orphanet

ORPHA: 447731
Klassifiseringsnivå: Disorder
ICD-10: D81.8

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