Diversity and combinations of infectious agents in 38 adults with an infection-triggered reactive haemophagocytic syndrome: a multicenter study

Abstract

Reactive haemophagocytic syndrome (HS) is a rare condition that occurs in patients with infections, haematological 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-centre case series. Here, we sought to characterize infectious triggers in a large, multicentre cohort of patients with HS. 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 patients with HS 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 years. Seven patients were presumed to be immunocompetent (18.4%), whereas 19 patients (50%) were infected with human immunodeficiency virus and 12 patients (31.6%) were immunocompromised for other reasons. Twenty-seven patients (71.1%) had a single infection, whereas six (15.8%) and five (13.1%) patients had, respectively, two and three concomitant infections. We observed pyogenic bacterial infections (n = 7), tuberculosis (n = 10), non-tuberculous mycobacteriosis (n = 3), viral infections (n = 17: 11 cytomegalovirus, three Epstein-Barr virus, two human herpesvirus 8, one herpes simplex virus 2), parasitic infections (n = 8: four disseminated toxoplasmosis, one leishmaniasis, three malaria), fungal infections (n = 5: four pulmonary pneumocystosis and one candidaemia). 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 malignancy-associated HS, patients with infection-triggered HS were younger and more likely to be immunocompromised, and had a better outcome.

Hemophagocytic syndrome in patients infected with the human immunodeficiency virus: A study of 15 consecutive patients

“…In another group of patients, principally those with late diagnosis (starting CD4 count of <350 cells/μL), erature to make comparisons. The number of patients included in the different case series ranges between 2 and 43, with the majority of studies being single-center [18][19][20][21][22][23][24][25][26][27]. In our series, the ratio of males to females was 2.75:1 and the mean age was 42 years, similar to those indicated in the literature, although males were less predominant (4:1 over-all).…”

“…Consequently, the risk practices for acquiring HIV infection were different and probably reflected local epidemiological patterns [18,19,22,23]. In general, severe immunosuppression (<200 CD4/ μL) and a detectable viral load at the time of diagnosis were common, although in our series and also in the literature, some cases of HPS presented higher CD4 values and undetectable viral loads [18][19][20][21][22][23][24][25][26][27]. Approximately half the patients were not in receipt of antiretroviral treatment, although the remainder were being treated [18,[23][24][25].…”

Prognostic Factors of Death in 151 Adults With Hemophagocytic Syndrome: Etiopathogenically Driven Analysis

“… 22 However, some descriptive studies have reported higher mortality rates in patients presenting with bacterial infections 13 , 23 , 24 or co-infections. 21 , 25 Our study reports, for the first time, that both the variety and number of infectious triggers implicated in HLH have a considerable influence on survival using time-to-event statistical models; the best survival curve was found for patients with parasitic/fungal infections, followed by those with viral infections, whereas the worst survival curves were for those with bacterial infections and multiple microbiological infections (of whom 71% also had bacterial infections in association with other microorganisms). Leishmaniasis, an endemic infection in Spain, was the most frequent parasitic infection related to HLH (8 cases, all born in Spain, of which 7 were reported from Madrid).…”

Disseminated toxoplasmosis associated with hemophagocytic syndrome after kidney transplantation: A case report and review

“…Among the parasitic and fungal infections, babesiosis, leishmaniosis, toxoplasmosis, malaria, strongyloidiasis, and pneumocystis agents were reported to be associated with HPS . Between 1994 and 2018, 17 cases in the literature described toxoplasmosis associated with HPS mostly in immunodeficient patients, including 10 HIV‐infected patients, 1 bone marrow transplant patient, 5 kidney transplant patients, and 2 immunocompetent patients . The rare association between disseminated toxoplasmosis and HPS in kidney recipients is only described in 5 cases with a mortality rate of 60%, including one reactivation case (Table ).…”

“…In contrast to the favorable course of toxoplasmosis in immunocompetent patients, disseminated toxoplasmosis is life‐threatening in immunocompromised patients . In rare cases, this acute infection could also be associated with hemophagocytic syndrome (HPS) . Donor‐to‐host transmission of toxoplasmosis is well known in heart transplantation but less frequent in kidney transplantation.…”