Lymphoproliferative Disease Associated with Primary Immune Disorder

**Semantic type:** Disease or Syndrome

**Definition:** A lymphoid proliferation that arises in the setting of immune deficiency due to a primary immunodeficiency or immunoregulatory disorder. The primary immune disorders most frequently associated with lymphoproliferative disorders are ataxia-telangiectasia, Wiskott-Aldrich syndrome, common variable immunodeficiency, severe combined immunodeficiency, X-linked lymphoproliferative disease, Nijmegen breakage syndrome, hyper-IgM syndrome, and autoimmune lymphoproliferative syndrome. (WHO 2017)

**Synonyms:** - Lymphoproliferative Disease Associated with PID