Exploring Pediatric Secondary Hemophagocytic Lymphohistiocytosis: An In-Depth Review of Causes, Diagnostic Methods, and Treatment Options - Takeaways - MDSpire

Exploring Pediatric Secondary Hemophagocytic Lymphohistiocytosis: An In-Depth Review of Causes, Diagnostic Methods, and Treatment Options

  • By

  • Ping Liu

  • Min Wang

  • Chuanwei Ban

  • Yumeng Ma

  • Juan Wang

  • Xin Lv

  • April 24, 2026

  • 0 min

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  • 1

    Pediatric secondary hemophagocytic lymphohistiocytosis (sHLH) primarily affects children under 3 years, with a median diagnosis age of 2.67 years.

  • 2

    Infection-associated HLH accounts for 78.2% of cases, with Epstein-Barr virus being the most common trigger in 57.3% of patients.

  • 3

    Common clinical manifestations include fever, lymphadenopathy, splenomegaly, and hepatomegaly, with respiratory involvement in over half of the patients.

  • 4

    The HLH-94/04 chemotherapy protocol was administered to 44.5% of patients, achieving a remission rate of 91.9% among those with EBV infection.

  • 5

    The overall in-hospital mortality rate for pediatric sHLH was 13.6%, with multi-organ dysfunction syndrome accounting for 73.3% of fatalities.

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