Hemophagocytic Lymphohistiocytosis in Children: Clinical Profile and Outcome
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Published on: November 21, 2018
Anna C Simon1, Chinnaiah Govindhareddy Delhi Kumar1, Debdatta Basu2
1Departments of Pediatrics.
Insights
Infections, particularly tropical agents, frequently trigger hemophagocytic lymphohistiocytosis (HLH) in South Indian children. While steroids can treat mild secondary HLH, primary HLH has a high mortality rate without stem cell transplant.
Area of Science:
- Pediatrics
- Hematology
- Infectious Diseases
Background:
- Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome.
- Understanding the clinical and etiological profile of HLH in specific geographic regions is crucial for timely diagnosis and management.
Purpose of the Study:
- To describe the clinical features, causes, and outcomes of pediatric HLH cases in a tertiary care hospital in South India.
- To identify common triggers and assess treatment responses and mortality rates.
Main Methods:
- A combined prospective (2-year) and retrospective (5-year) descriptive study.
- Inclusion of children (birth to 18 years) meeting HLH-2004 diagnostic criteria.
- Analysis of patient records for clinical data, triggers, complications, treatment, and outcomes.
Main Results:
- Fifty-three children with HLH were analyzed; 62% had infectious triggers, predominantly bacterial and viral.
- Scrub typhus and dengue were the most common infectious agents identified.
- Common presentations included fever, hepatomegaly, anemia, and hyperferritinemia; mortality was 41%.
Conclusions:
- Infections, especially tropical agents, are the primary drivers of HLH in this cohort.
- Steroid monotherapy may suffice for mild secondary HLH, but primary HLH necessitates aggressive treatment like stem cell transplant due to high mortality.
Objectives:
The objectives of this study were to describe the clinical and etiologic profile and outcomes of children with hemophagocytic lymphohistiocytosis (HLH) in a tertiary care hospital in South India.
Methods:
This is a combined 2-year prospective (2017 to 2018) and 5-year retrospective (2012 to 2016) descriptive study in which children from birth to 18 years who satisfied the HLH-2004 diagnostic criteria were included. Case details from patient records were analyzed.
Results:
Fifty-three cases were enrolled of which 20 were prospective and 33 were retrospective. Fever, hepatomegaly, anemia, and hyperferritinemia were the common presentations. Infectious triggers were found in 33 (62%) cases. Five cases were secondary to rheumatic diseases, and 8 were primary HLH. Bacterial (14 cases) followed by viral infections (10 cases) were the leading triggers. Scrub typhus (6 cases) and dengue (4 cases) were the most common infectious agents. Major complications include febrile neutropenia (38%) and multiorgan dysfunction (26%). One child developed secondary malignancy. The most frequently used immunosuppressive drug for the treatment of HLH was steroid (70%), while 28% of cases recovered with only supportive therapy. The overall mortality was 41%.
Conclusions:
Infections were the most common triggers for HLH of which tropical infectious agents constituted the majority. Treatment with steroids alone or regimens without cytotoxic drugs may result in resolution of secondary HLH with mild to moderate disease activity. Without stem cell transplant, primary HLH has a high mortality rate.
