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WHO Classification of Tumours
Lymphoproliferative diseases associated with primary immune disorders
Tumours of haematopoietic and lymphoid tissues


Definition

Lymphoproliferative diseases (LPD) associated with primary immune disorders (PID) are lymphoid proliferations that arise as a result of immune deficiency due to a primary immunodeficiency or immuno­regulatory disorder. Because the pathology and pathogenesis of the more than 60 PID are heterogeneous, the manifestations of the lymphoproliferative diseases are highly variable. The PID most frequently associated with LPD are ataxia telangiectasia (AT), Wiskott-Aldrich syndrome (WAS), common variable immunodeficiency (CVID), severe combined immunodeficiency (SCID), X-linked lymphoproliferative disorder (XLP), Nijmegen breakage syndrome (NBS), hyper-IgM syndrome and autoimmune lymphoproliferative syndrome (ALPS).