Please cite this article in press as: Sánchez-Moreno P, et al. Typhoid fever causing haemophagocytic lymphohistiocyto- sis in a non-endemic country – first case report and review of the current literature. Enferm Infecc Microbiol Clin. 2017. https://doi.org/10.1016/j.eimc.2018.04.011 ARTICLE IN PRESS G Model EIMC-1854; No. of Pages 5 Enferm Infecc Microbiol Clin. 2017;xxx(xx):xxx–xxx www.elsevier.es/eimc Brief report Typhoid fever causing haemophagocytic lymphohistiocytosis in a non-endemic country – first case report and review of the current literature Paula Sánchez-Moreno a , Peter Olbrich a,∗ , Lola Falcón-Neyra a , Jose Manuel Lucena b , Javier Aznar c , Olaf Neth a a Paediatric Infectious Diseases, Rheumatology and Immunology Unit, Hospital Universitario Virgen del Rocío, Institute of Biomedicine of Seville (IBIS), Seville, Spain b Department of Immunology, Institute of Biomedicine of Seville (IBIS), Seville, Spain c Clinical Unit of Infectious Diseases, Microbiology, and Preventive Medicine, Institute of Biomedicine of Seville (IBIS), Hospital Universitario Virgen del Rocío, University of Seville, Seville, Spain a r t i c l e i n f o Article history: Received 24 January 2018 Accepted 13 April 2018 Available online xxx Keywords: Typhoid fever Salmonella typhi Haemophagocytic lymphohistiocytosis a b s t r a c t Introduction: Development of secondary haemophagocytic lymphohistiocytosis (sHLH) in the context of typhoid fever (TF) is a very rare but serious complication. Methods: Description of the first pediatric case of typhoid fever acquired in a non-endemic area compli- cated by sHLH. A systematic literature review of sHLH in the context of TF was performed with extraction of epidemiological, clinical and laboratory data. Results: The literature search revealed 17 articles (22 patients). Fifteen patients were eligible for data analysis (53.4% children). All patients had fever and pancytopenia. Transaminases and LDH were fre- quently elevated (46.6%). Salmonella typhi was detected mainly by blood culture (64.3%). All the patients received antibiotics whereas immunomodulation (dexamethasone) was used in two cases. Conclusions: A high suspicion index for this condition is needed even in non-endemic areas. The addition of immunmodulation to standard antimicrobial therapy should be considered in selected cases. © 2018 Elsevier Espa ˜ na, S.L.U. and Sociedad Espa ˜ nola de Enfermedades Infecciosas y Microbiolog´ ıa Cl´ ınica. All rights reserved. Fiebre tifoidea como causa de linfohistiocitosis hemofagocítica en un país no endémico: primer caso reportado y revisión de la literatura Palabras clave: Fiebre tifoidea Salmonella typhi Linfohistiocitosis hemofagocítica r e s u m e n Introducción: El síndrome hemofagocítico (HLH) secundario en el contexto de fiebre tifoidea es una complicación rara pero seria. Métodos: Descripción del primer caso pediátrico de fiebre tifoidea adquirida en área no endémica com- plicada con síndrome hemofagocítico y revisión sistemática de la literatura de casos de HLH secundarios a fiebre tifoidea. Descripción de datos epidemiológicos, clínicos y de laboratorio, diagnóstico y manejo. Resultados: La búsqueda bibliográfica reveló 17 artículos (22 pacientes). Quince pacientes eran elegibles para el análisis (53,4% ni˜ nos). La fiebre y la pancitopenia estaban siempre presentes, y las transaminasas y la LDH estaban frecuentemente elevados (46,6%). La detección de S. typhi se realizó mediante hemocultivo, principalmente (64,3%). Todos los pacientes reportados recibieron antibióticos; la dexametasona fue usada como tratamiento inmunomodulador en 2 de los casos. ∗ Corresponding author. E-mail address: olbpet@gmail.com (P. Olbrich). https://doi.org/10.1016/j.eimc.2018.04.011 0213-005X/© 2018 Elsevier Espa ˜ na, S.L.U. and Sociedad Espa ˜ nola de Enfermedades Infecciosas y Microbiolog´ ıa Cl´ ınica. All rights reserved.