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Infection associated hemophagocytic syndrome: a report of 50 children
1Hematology Division, Department of Pediatrics, Queen Sirikit National Institute of Child Health, Bangkok 10400, Thailand.
Insights
Infection-associated hemophagocytic syndrome (IAHS) in children has a high mortality rate of 70%. Aggressive supportive care and treatment of underlying infections are crucial for managing this severe condition.
Area of Science:
- Pediatrics
- Infectious Diseases
- Hematology
Background:
- Infection-associated hemophagocytic syndrome (IAHS) is a life-threatening condition characterized by excessive immune activation.
- IAHS can occur in children following various infections, leading to significant morbidity and mortality.
Purpose of the Study:
- To describe the clinical characteristics, treatment, and outcomes of children diagnosed with IAHS.
- To identify factors associated with mortality in pediatric IAHS patients.
Main Methods:
- Retrospective analysis of 50 children diagnosed with IAHS between 1988 and 1997.
- Review of patient demographics, associated infections, treatment modalities, and outcomes.
Main Results:
- The study identified 50 pediatric cases of IAHS, with a mean age of 4.14 years.
- A high mortality rate of 70% was observed, primarily due to coagulopathy, multiple organ failure, and opportunistic infections.
- Two patients developed acute lymphoblastic leukemia post-recovery.
Conclusions:
- IAHS in children is associated with a poor prognosis, necessitating prompt diagnosis and aggressive management.
- Effective treatment strategies should focus on controlling the underlying infection and providing intensive supportive care.
Abstract:
Fifty children were diagnosed with infection associated hemophagocytic syndrome (IAHS) over the 10 year period from January 1988 through December 1997 at Queen Sirikit National Institute of Child Health, Thailand. Their ages ranged from 2 months to 14 years (mean 4.14 years). There was no difference in sex. Bacterial, mycobacterial, viral, fungal and protozoa were the associated infections in some of these patients. Supportive with specific therapy for the underlying disease was administered aggressively in all patients. Intravenous immuneglobulin (IVIG) was given in 8 patients. Thirty-five patients (70%) died, mostly as a result of coagulopathy with multiple organ failure and opportunistic infections. Two patients developed acute lymphoblastic leukemia 25 days and 3 months after recovering from IAHS.
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