HEMOPHAGOCYTIC-SYNDROME IN PATIENTS INFECTED WITH THE HUMAN-IMMUNODEFICIENCY-VIRUS - 9 CASES AND A REVIEW

We report nine cases of haemophagocytic syndrome (HS) in patients with human immunodeficiency virus infection, and review the 17 cases descr ibed to date in the literature. In 21 of 26 cases, HS developed during an advanced stage of immunodeficiency. Clinical and haematological si gns are not specific in this setting, and the diagnosis relies on hist ological features, mainly bone marrow examination. An opportunistic in fection was associated in three cases and a lymphoid malignancy in two of our nine cases. The role of the human immunodeficiency virus in th e occurrence of the HS remains to be defined. The overall prognosis is poor as six of our nine patients died within 12 months of presentatio n.