Outcome and Prognostic Factors of Hemophagocytic Lymphohistiocytosis in Children: Experience From a Low- and Middle-Income Country

TL;DR

This study examines the outcomes and factors affecting survival in children with HLH in a low- and middle-income country, emphasizing the importance of early diagnosis and treatment.

Contribution

The study provides insights into HLH prognosis and treatment in a resource-limited setting, highlighting the role of early referral and HSCT.

Findings

Overall survival at two, five, and 10 years was 38%, 37%, and 36.1%, respectively.

HSCT cured 65.4% of the 26 patients who underwent the procedure.

Delayed diagnosis and misdiagnosis were identified as major contributors to high mortality in HLH cases.

Abstract

Objective

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition especially in low- and middle-income countries (LMICs). This study was done to evaluate the outcome and prognostic factors of HLH in patients presenting to our center.

Methods

The study was carried out at the Paediatric Oncology Department of Combined Military Hospital (CMH) in Rawalpindi, Pakistan. All cases of HLH, from one month to 15 years of age enrolled between January 1, 2013 to June 30, 2023, were included. IBM SPSS Statistics for Windows, version 25.0 (released 2017, IBM Corp., Armonk, NY) was used for statistical analysis, and t-test and chi-square tests were used for comparison between continuous and categorical variables. Frequencies and percentages were calculated for categorical variables.

Results

Out of 115 patients, seven (6%) abandoned the treatment. The data of 108 cases, including 58 males (53.7%), were analyzed. The mean age at diagnosis was 31.5 ± 39.03 months. The mean time to reach a pediatric oncologist was 30.20 ± 22.15 days. Fever and pallor were common symptoms occurring in 107 (99.1%) and 98 (90.7%) cases, respectively. Jaundice was present in 44 (40.7%), visceromegaly in 64 (59.3%), and bruising/bleeding in 16 cases (14.8%). Twenty-six (24.1%) patients underwent hematopoietic stem cell transplant (HSCT), out of which 17 (65.4%) children were cured. Overall survival at two years, five years, and 10 years was 38%, 37%, and 36.1%, respectively. Disease-free survival at two years, five years, and 10 years was 33.3%, 32.4%, and 31.5%, respectively.

Conclusion

HLH leads to high mortality due to delayed or misdiagnosis in LMICs. Early diagnosis and early referral to a pediatric oncologist is the detrimental factor in survival for HLH. HSCT is the treatment of choice for primary, refractory, or relapse cases.

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