Background: The hemophagocytic syndrome has previously been reported in different infectious diseases (EBV, CMV, tuberculosis...) but rarely in adults with AIDS and never in children suffering from AIDS.
Case reports: A hemophagocytic syndrome was recognized during the follow-up of 3 children with AIDS. The first, a 9-year-old girl developed an acute EBV coinfection and was treated with shots of corticosteroids and vepesid but died shortly afterwards. The second patient, a 3-year-old girl was infected with Aspergillus fumigatus for which she was given amphotericin B with a rapid improvement. The third patient, an 8-year-old boy had multi-resistant Streptococcus pneumoniae otitis and pneumonitis; his condition improved rapidly with adapted antibiotherapy.
Discussion: The HIV-hemophagocytic syndrome is not exceptional in HIV infection because of the association of immunodeficiency and resulting superinfections. Its diagnosis and treatment should be etiologic. Severe cases without etiology could benefit from chemotherapy.
Conclusion: Management and outcome of this potentially lethal syndrome might depend on the identification of a curable infectious cause.