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This paper focuses on primary immune regulatory disorders including autoimmune lymphoproliferative syndrome (ALPS) and ALPS\u2010like syndromes, immunodysregulation, polyendocrinopathy, enteropathy, X\u2010linked (IPEX) and IPEX\u2010like disorders, common variable immunodeficiency (CVID), CVID\u2010like, and late\u2010onset combined immunodeficiency (CID) disorders. Hyperinflammatory disorders and those associated with increased susceptibility to lymphoid malignancies are also discussed. 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