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Scrub Typhus-Associated Hemophagocytic Lymphohistiocytosis: Not a Rare Entity in Pediatric Age Group
Ankika Basu1, Satyabrata Roy Chowdhoury1, Mihir Sarkar1
1Department of Pediatrics, Medical College and Hospital, Kolkata, West Bengal 700073, India.
Insights
Scrub typhus-associated hemophagocytic lymphohistiocytosis (HLH) in children is increasingly recognized. Younger age, prolonged fever, and complications like ARDS and MODS are key predictors, with doxycycline treatment often effective.
Area of Science:
- Pediatric Infectious Diseases
- Hematology
- Immunology
Background:
- Scrub typhus is a significant cause of febrile illness in children.
- Hemophagocytic lymphohistiocytosis (HLH) is a severe, life-threatening hyperinflammatory syndrome.
- Understanding scrub typhus-associated HLH in pediatric patients is crucial for timely diagnosis and management.
Purpose of the Study:
- To investigate the demographic, clinical, and laboratory characteristics of pediatric patients with scrub typhus-associated HLH.
- To compare the outcomes of HLH patients with non-HLH scrub typhus patients.
- To identify clinical predictors of HLH in children with scrub typhus.
Main Methods:
- A prospective observational study was conducted over one year in a tertiary care hospital.
- Pediatric patients (1 month to 12 years) with confirmed scrub typhus (IgM ELISA positive) were enrolled.
- HLH diagnosis was based on the HLH-2004 criteria, with detailed clinical and laboratory data collected.
Main Results:
- Out of 58 scrub typhus cases, 18 (31%) had HLH. All HLH patients presented with anemia, thrombocytopenia, and hyperferritinemia.
- Complications such as acute respiratory distress syndrome (ARDS) and multiple organ dysfunction syndrome (MODS) were significantly higher in the HLH group.
- Clinical predictors for HLH included younger age (<3 years), prolonged fever (>7 days), convulsions, ARDS, and MODS.
Conclusions:
- Scrub typhus-associated HLH is an emerging concern in pediatric populations.
- Clinicians should be vigilant for HLH in children with scrub typhus, especially those with risk factors like prolonged fever and severe complications.
- Primary treatment of scrub typhus is paramount, and immunomodulatory therapy may not always be necessary.
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.
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