LYMPHOPROLIFERATIVE SYNDROME, X-LINKED, 1

Abdomen
Fulminant hepatitis
Liver failure
Gastrointestinal
Hepatomegaly
Splenomegaly
General
Reduced life expectancy, death by 10 years of age in 70% of patients
Hematologic
Anemia
Atypical lymphocytosis
Pancytopenia
Thrombocytopenia
Immunologic System
Combined variable immunodeficiency involving B and T cells
Fulminant infectious mononucleosis
Histology shows large regions of necrosis in lymph nodes, thymus, bone marrow, and spleen
Lymphadenopathy
Normal number of B cells
Normal number of T cells
Reduced CD4+/CD8+ ratio with CD8+ predominance
Reduced natural killer cell activity
Metabolic Abnormality
Increased IgM levels
Reduced IgG levels
Neoplasia
Hemophagocytic lymphohistiocytosis
Lymphoma
Neurologic Abnormality
Encephalitis
Hepatic encephalopathy
Meningitis
Respiratory
Lymphoid granulomatosis
Pharyngitis