bare lymphocyte syndrome type I

bare lymphocyte syndrome type I

An extremely rare disorder (9 cases in the world literature), which is more banal than bare lymphocyte syndrome types II or III, characterised by chronic bacterial infections often beginning in the first decade of life that are restricted to the respiratory tract and nasal sinuses, often accompanied by nasal polyps.

Molecular pathology
Defects of TAP1, TAP2 or TAPBP cause bare lymphocyte syndrome type I.