Q fever and Mediterranean spotted fever associated with hemophagocytic syndrome: case study and literature review

Abstract

Background: Hemophagocytosis during Q fever (QF) and Mediterranean spotted fever (MSF) is rare and only a few cases have been reported. We aimed to investigate the characteristics, outcome, and treatment of QF/MSF-associated hemophagocytosis.

Methods: We retrospectively reviewed all patients with a diagnosis of QF or MSF and suspected hemophagocytic syndrome (HS), according to Henter's criteria, between 2002 and 2011, and compared the latter to patients without HS or with lymphoma-associated HS.

Results: Seventeen patients with HS (median age 42 years, range 5-68 years; five females (29%)) with QF (n=8) and MSF (n=9) were included in this study. When comparing patients with QF- and MSF-associated HS with patients without HS (n=11), HS-associated signs (splenomegaly, ferritinemia, hypertriglyceridemia, and cytopenia) were significantly more frequent in patients with histological HS (p<0.05), along with a greater number of Henter's criteria. Despite the presence of HS-associated signs, treatment was similar in these two subgroups, including the time to recovery and the outcome. When compared to lymphoma-associated HS (n=10), the outcome in QF/MSF-associated HS was significantly different, with mortality in 70% of lymphoma patients versus none in QF- and MSF-associated HS (p<0.05).

Conclusion: Hemophagocytosis is a rare occurrence during the course of QF and MSF. The presence of profound cytopenia is quite unusual in QF and MSF and should bring to mind the presence of associated HS. Nevertheless, hemophagocytic syndrome is associated with a good outcome in this condition.