Rare diseases · Sign or symptom

Abnormal double-negative T cell proportion

HP:0031399

What it means

Abnormal increase or decrease of double-negative (DN) CD3+CD4-CD8- T cells, measured either as percentage of total CD3+ T cells in the blood, or as absolute number per microliter of blood, compared to a reference range for a given sex and age-group. These are usually measured within the TCR alpha/beta positive population.

T lymphocytes bearing the T cell receptor (TCR) but lacking CD4, CD8, and markers of natural killer (NK) cell differentiation, i.e., TCR(+)CD3(+)CD4(-)CD8(-), are known as double-negative (DN) T cells and are defined by their capability of inhibiting immune responses via directly killing effector T cells in an antigen specific fashion. Furthermore, DN Treg cells have been shown to develop regulatory activity after encountering specific antigens, partially mediated by the acquisition of MHC-peptide complexes from antigen presenting cells (APCs).

Part of the broader category

Also called: Abnormal CD4-negative, CD8-negative regulatory T cell distribution · Abnormal DN Treg distribution · Abnormal Double negative Treg distribution · Abnormal double-negative regulatory T cell distribution · Abnormal proportion of double-negative regulatory T cell

Abnormal double-negative T cell proportion

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This page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.