systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
A rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever and malaise, hepatosplenomegaly with or without lymphadenopathy, liver failure, severe pancytopenia and a rapid progression towards multi-organ failure and hemophagocytic syndrome with a fatal issue. It is characterized by clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype.
MONDO:0000430
mature T-cell and NK-cell non-Hodgkin lymphoma
is_a
MONDO:0000621
immune system cancer
is_a
MONDO:0003659
pediatric lymphoma
is_a
MONDO:0006188
EBV-positive T-cell lymphoproliferative disorder of childhood
is_a
MONDO:0017343
Epstein-Barr virus-associated lymphoproliferative disorder
is_a
EBV-positive T-cell lymphoproliferative disorder of childhood
broad
systemic EBV+ T-cell LPD of childhood
exact
systemic EBV-positive T-cell lymphoma of childhood
exact
systemic EBV-positive T-cell lymphoproliferative disease of childhood
exact
systemic EBV-positive T-cell lymphoproliferative disorder of childhood
exact