Disease - Lymphoproliferative syndrome 1
Definition
A rare immunodeficiency characterized by extreme susceptibility to infection with Epstein-Barr virus (EBV). Inadequate immune response to EBV can have a fatal outcome. Clinical features include splenomegaly, lymphadenopathy, anemia, thrombocytopenia, pancytopenia, recurrent infections. There is an increased risk for lymphoma.
Acronym
LPFS1
Synonyms
Lymphoproliferative syndrome, EBV-associated, autosomal, 1
Disclaimer
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