<unk>  <unk> , is a form of lymphoproliferative disorder (LPDs). It affects lymphocyte apoptosis. It is a rare genetic disorder of abnormal lymphocyte survival caused by defective Fas mediated apoptosis. Normally, after infectious insult, the immune system down-regulates by increasing Fas expression on activated B and T lymphocytes and Fas-ligand on activated T lymphocytes. Fas and Fas-ligand interact to trigger the caspase cascade, leading to cell apoptosis. Patients with<unk>  have a defect in this apoptotic pathway, leading to chronic non-malignant lymphoproliferation,<unk>  disease, and secondary cancers.
Autoimmune lymphoproliferative syndrome
Diffuse infiltrative lymphocytosis syndrome
Thymoma with immunodeficiency
Bare lymphocyte syndrome
X-linked lymphoproliferative disease
Thymoma-associated multiorgan autoimmunity
Adult-onset immunodeficiency syndrome
Epstein–Barr virus–associated lymphoproliferative diseases