Enteric fever is one of the important causes of tropical fever with a prevalence of 11-21 million cases worldwide annually. It encompasses both typhoid and paratyphoid fever. Salmonella enterica Typhi is the causative organism for typhoid fever, manifesting as an uncomplicated febrile illness to life-threatening sepsis with multiorgan dysfunction. It is complicated by neuropsychiatric manifestation (2%-40%), gastrointestinal bleeding (10%), and intestinal perforation (1%-3%). Hemophagocytic Lymphohistiocytosis (HLH) is a rare complication of typhoid fever. Herein we report the case of a 24-year-old male from North-Eastern India, presenting with spikes of fever, altered behavior, a generalized rash, haematochezia, and hemophagocytes on bone marrow examination due to hemophagocytic lymphohistiocytosis secondary to complicated typhoid fever.

Secondary hemophagocytic lymphohistiocytosis (sHLH) triggered by Salmonella enterica serovar Typhimurium is rare in pediatric patients. There is no consensus on how to treat S. typhimurium-triggered sHLH.

A 9-year-old boy with intermittent fever for 3 d presented to our hospital with positive results for S. typhimurium, human rhinovirus, and Mycoplasma pneumoniae infections. At the time of admission to our institution, the patient's T helper 1/T helper 2 cytokine levels were 326 pg/mL for interleukin 6 (IL-6), 9.1 pg/mL for IL-10, and 246.7 pg/mL for interferon-gamma (IFN-γ), for which the ratio of IL-10 to IFN-γ was 0.04. In this study, the patient received meropenem, linezolid, and cefoperazone/sulbactam in combination with high-dose methylprednisolone therapy (10 mg/kg/d for 3 d) and antishock supportive treatment twice. After careful evaluation, this patient did not receive HLH chemotherapy and recovered well.

S. Typhimurium infection-triggered sHLH patient had a ratio of IL-10 to IFN-γ ≤ 1.33, an IL-10 concentration ≤ 10.0 pg/mL, and/or an IFN-γ concentration ≤ 225 pg/mL at admission. Early antimicrobial and supportive treatment was sufficient, and the HLH-94/2004 protocol was not necessary under these conditions.

Pediatric hemophagocytic syndrome (HS) is a severe and often fatal clinical disorder. This syndrome is frequently unrecognized, and thus, affected children may receive suboptimal management, leading to an increase in mortality. The purpose of this review is to provide a clinical guide to (1) the recognition of HS based on clinical, biologic, and pathologic features; (2) the identification of the primary cause of HS in a given affected child; and (3) the initiation of effective treatment in a timely manner.