| X-linked lymphoproliferative syndrome |
Epstein-Barr virus infection; fatal infectious mononucleosis associated with X-linked lymphoproliferative syndrome; acquired hypogammaglobulinemia; dysgammaglobulinemia; malignant lymphoma; pancytopenia; vasculitis; lymphomatoid granulomatosis; hepatitis; pharyngitis; hepatomegaly; fulminant hepatitis; hepatic failure; splenomegaly; meningitis; encephalitis; hepatic encephalopathy; Aplastic anemia; thrombocytopenia; atypical lymphocytosis; lymphadenopathy; combined variable immunodeficiency involving B and T cells; normal number of B cells; normal number of T cells; reduced CD4+/CD8+ ratio with CD8+ predominance; reduced natural killer cell activity; fulminant infectious mononucleosis; histology shows large regions of necrosis in lymph nodes, thymus, bone marrow, and spleen; hemophagocytic lymphohistiocytosis; decreased IgG; increased IgM level; Low to absent NKT cells |
Diseases of Immune Dysregulation |
X-linked recessive |