Haemophagocytic syndrome associated with infections: Not so uncommon

F F Rodríguez Vidigal; A Calvo Cano; M Sánchez Sánchez; N Nogales Muñoz; A Vera Tomé; A Muñoz Sanz

doi:10.1016/j.rce.2019.04.008

Haemophagocytic syndrome associated with infections: Not so uncommon

Rev Clin Esp (Barc). 2020 Mar;220(2):109-114. doi: 10.1016/j.rce.2019.04.008. Epub 2019 Jun 13.

[Article in English, Spanish]

Authors

F F Rodríguez Vidigal¹, A Calvo Cano¹, M Sánchez Sánchez¹, N Nogales Muñoz¹, A Vera Tomé¹, A Muñoz Sanz²

Affiliations

¹ Servicio de Patología Infecciosa, Hospital Universitario de Badajoz. Universidad de Extremadura, Badajoz, España.
² Servicio de Patología Infecciosa, Hospital Universitario de Badajoz. Universidad de Extremadura, Badajoz, España. Electronic address: oyacoi@unex.es.

PMID: 31202502
DOI: 10.1016/j.rce.2019.04.008

Abstract

Background: Haemophagocytic syndrome (HPS) is a severe immunological disorder characterised by uncontrolled inflammation and multiple organ failure. HPS can be triggered by viral, bacterial, fungal and parasitical infections. We report our experience with infection-related HPS and estimate its local incidence.

Material and method: We conducted an observational retrospective study of infection-associated HPS in patients treated in the Department of Infectious Diseases of a university hospital within a 5-year period, as well as a review of the published series in Europe.

Results: HPS was associated with infection by cytomegalovirus in 2 women with Crohn's disease and was associated with visceral leishmaniosis in 4 patients (3 men, 1 woman; 1 case of multiple myeloma; 2 cases of solid tumours; 1 case of no apparent disease). Two patients died, and the estimated incidence rate was 0.58/100,000 inhabitants/year. The published series are mixed.

Conclusions: Infection-related HPS must be more common than reported. The geographical environment can influence the triggering infections (in our environment, Leishmania should be considered).

Keywords: Citomegalovirus; Cytomegalovirus; Desencadenante; Haemophagocytic lymphohistiocytosis; Haemophagocytic syndrome; Infección; Infection; Leishmania; Linfohistiocitosis hemofagocítica; Síndrome hemofagocítico; Trigger.