Scrub Typhus-Associated Hemophagocytic Lymphohistiocytosis: Not a Rare Entity in Pediatric Age Group

Abstract

Abstract Background Our goal was to study the demographic, clinical and laboratory profile and outcome of scrub typhus-associated hemophagocytic lymphohistiocytosis (HLH) in the pediatric age group. Methods We conducted a prospective observational study in a tertiary care teaching hospital over a period of 1 year. Children in the age group of 1 month to 12 years with IgM ELISA positive for scrub typhus were included in the study. HLH was diagnosed using HLH-2004 criteria. Demographic, clinical and laboratory profile, treatment and outcome of HLH patients were noted and also compared with non-HLH scrub typhus patients. Results Among 58 children with scrub typhus infection, 18 had HLH. The mean age of patients with HLH was 35.3 ± 44.8 months and 61% were male. Anemia, thrombocytopenia and hyperferritinemia were seen in all the patients. Hypertriglyceridemia, hypofibrinogenemia and coagulopathy were noted in 78%, 56% and 44%, respectively. All the patients were treated with intravenous doxycycline for an average duration of 9.5 days. Intravenous immunoglobulin and methylprednisolone were given in 33% and 22% cases, respectively. Complications like acute respiratory distress syndrome (ARDS) (p = 0.001) and MODS (p = 0.004) were significantly high in the HLH group. Younger age (<3 years), fever > 7 days, presence of convulsion, ARDS and MODS were the clinical predictors of scrub typhus-associated HLH. Conclusion HLH in scrub typhus infected children is being increasingly recognized. Younger age, prolonged fever, presence of convulsion, ARDS and MODS should alert clinicians of the risk of HLH. Treating the primary cause usually cures the disease and immunomodulator therapy need not be routinely administered.