These characteristics have implicated KIR3DL2 in several pathologies: ankylosing spondylitis and cutaneous T-cell lymphomas such as Sézary syndrome, CD30<sup>+</sup> cutaneous lymphoma, and transformed mycosis fungoides.
KIR-3DL2+CD4+ T cells in patients with ankylosing spondylitis were oligoclonal and enriched for markers of T cell activation and for the gut homing receptor CCR9.
Increased expression of HC10-reactive molecules on AS monocytes was seen, supporting the possible role of the KIR3DL2/B272 pair in the pathogenesis of AS.
KIR3DL2 binds to HLA-B27 dimers and free H chains more strongly than other HLA class I and promotes the expansion of T cells in ankylosing spondylitis.