EBV-associated Hodgkin's disease in an HIV-infected child presenting with a hemophagocytic syndrome

An 8-years-old boy was admitted with fever of unknown origin, cervical lymp hadenopathy and hepatosplenomegaly and weight loss. His mother's HIV infect ion was diagnosed two weeks before his hospitalization, so he was diagnosed as perinatally acquired A-EDS. Serology and serial cultures were negative for viral infections, toxoplasmosis, chagas, tuberculosis and atypical myco bacterium. The patient met clinical and laboratory criteria for hemophagocy tic syndrome (HS) that was confirmed on bone marrow aspirate and biopsy. A cervical lymph node biopsy was performed which was diagnosed as Hodgkin's d isease (HD) diffuse fibrosis lymphocyte depletion subtype. EBERs in situ hy bridization and LMP-1 immunohistochemistry on the lymph node biopsy establi shed the EBV association. On the basis of a sequence of appearance of the c linical, laboratory and histological signs, HIV, EBV or HD may have trigger ed HS as the last fatal event in this pediatric patient.