Diversity and combinations of infectious agents in 38 adults with an infection-triggered reactive hemophagocytic syndrome: a multicenter study.
Clin Microbiol Infect. 2015 Dec 11;
Authors: Lerolle N, Laanani M, Rivière S, Galicier L, Coppo P, Meynard JL, Molina JM, Azoulay E, Aumont C, Marzac C, Fardet L, Lambotte O
Reactive hemophagocytic syndrome (HS) is a rare condition that occurs in patients with infections, hematological malignancies or autoimmune diseases. Although various microorganisms are thought to trigger HS, most of the literature data on this topic have been gathered in single-center case series. Here, we sought to characterize infectious triggers in a large, multicenter cohort of HS patients. Patients were included in the present study if HS was solely due to one or more infections. Detailed microbiological data were recorded. Of the 162 HS patients in the cohort, 40 (25%) had at least one infection and 38 of the latter (including 14 women, 36.8%) were included. The median age was 46. Patients were presumed to be immunocompetent in 7 cases (18.4%), whereas 19 patients (50%) were infected with HIV and 12 patients (31.6%) were immunocompromised for other reasons. Twenty-seven patients (71.1%) had a single infection, whereas 6 (15.8%) and 5 patients (13.1%) had respectively 2 and 3 concomitant infections. We observed pyogenic bacterial infections (n=7), tuberculosis (n=10), nontuberculous mycobacteriosis (n=3), viral infections (n=17: 11 CMV, 3 EBV, 2 HHV8, 1 HSV2), parasitic infections (n=8: 4 cases of disseminated toxoplasmosis, 1 leishmaniasis, 3 malaria), fungal infections (n=5: 4 cases of pulmonary pneumocystosis and 1 candidemia). Eighteen patients (47.4%) received corticosteroids and/or etoposide. Twelve patients died (31.6%). All multiple infections and all deaths occurred in immunocompromised patients. When compared with patients suffering from malignancies-associated HS, patients suffering from infection-triggered HS were younger and more likely to be immunocompromised, and had a better outcome.
PMID: 26686809 [PubMed - as supplied by publisher]