Monarch Disease Ontology id MONDO_0024777 (immunodeficiency 98 with autoinflammation, X-linked) is an immunodeficiency disease characterized by onset of recurrent infections associated with lymphoproliferation and autoinflammation in the first decade of life. Mostly males are affected; carrier females may have mild symptoms. Laboratory studies show evidence of immune dysregulation, including hypogammaglobulinemia with reduced memory B cells, skewed T-cell subsets, increased levels of proinflammatory cytokines, activated T cells and monocytes, and autoimmune cytopenias, including neutropenia. Also known as: IMD98, X-linked immunodeficiency with autoinflammation, inflammation, neutropenia, bone marrow failure, and lymphoproliferation caused by TLR8.