Typhoid fever causing haemophagocytic lymphohistiocytosis in a non-endemic country - first case report and review of the current literature

Paula Sánchez-Moreno; Peter Olbrich; Lola Falcón-Neyra; Jose Manuel Lucena; Javier Aznar; Olaf Neth

doi:10.1016/j.eimc.2018.04.011

Typhoid fever causing haemophagocytic lymphohistiocytosis in a non-endemic country - first case report and review of the current literature

Enferm Infecc Microbiol Clin (Engl Ed). 2019 Feb;37(2):112-116. doi: 10.1016/j.eimc.2018.04.011. Epub 2018 Jun 7.

[Article in English, Spanish]

Authors

Paula Sánchez-Moreno¹, Peter Olbrich², Lola Falcón-Neyra¹, Jose Manuel Lucena³, Javier Aznar⁴, Olaf Neth¹

Affiliations

¹ Paediatric Infectious Diseases, Rheumatology and Immunology Unit, Hospital Universitario Virgen del Rocío, Institute of Biomedicine of Seville (IBIS), Seville, Spain.
² Paediatric Infectious Diseases, Rheumatology and Immunology Unit, Hospital Universitario Virgen del Rocío, Institute of Biomedicine of Seville (IBIS), Seville, Spain. Electronic address: [email protected].
³ Department of Immunology, Institute of Biomedicine of Seville (IBIS), Seville, Spain.
⁴ 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.

PMID: 29887216
DOI: 10.1016/j.eimc.2018.04.011

Abstract

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 complicated 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 frequently 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.

Keywords: Fiebre tifoidea; Haemophagocytic lymphohistiocytosis; Linfohistiocitosis hemofagocítica; Salmonella typhi; Typhoid fever.

Publication types

Case Reports
Review

MeSH terms

Abdominal Abscess / diagnosis
Abdominal Pain / etiology
Age Distribution
Appendicitis / diagnosis
Asia / epidemiology
Ceftriaxone / therapeutic use
Child
Developed Countries
Diagnosis, Differential
Endemic Diseases
Fever / etiology
Hepatomegaly / etiology
Humans
Lymphohistiocytosis, Hemophagocytic / diagnosis
Lymphohistiocytosis, Hemophagocytic / drug therapy
Lymphohistiocytosis, Hemophagocytic / epidemiology
Lymphohistiocytosis, Hemophagocytic / etiology*
Male
Methylprednisolone / therapeutic use
Middle East / epidemiology
Sex Distribution
Spain / epidemiology
Splenomegaly / etiology
Typhoid Fever / complications*
Typhoid Fever / diagnosis
Typhoid Fever / drug therapy
Typhoid Fever / epidemiology

Substances

Ceftriaxone
Methylprednisolone