Clinical analysis of five cases with pediatric secondary hemophagocytic lymphohistiocytosis / 中华实用儿科临床杂志

ABSTRACT

Objective:To analyze the clinical manifestations, laboratory examination characteristics and treatment of secondary hemophagocytic lymphohistiocytosis (HLH) in children with different etiologies, and to further explore the stratified diagnosis and treatment of pediatric secondary HLH, in an attempt to improve pediatricians′ understanding of this disease.Methods:The clinical data of 5 children with secondary HLH treated at Beijing Children′s Hospital, Capital Medical University from January 2018 to December 2019 were retrospectively analyzed, to explore the diagnosis and subsequent treatment of the primary HLH.Results:Five patients (including 3 boys and 2 girls) had a median onset age of 1.5 (0.5-12.7) years.At the early stage of the disease, all patients had fever and hemocytopenia.After admission, HLH-related examinations were performed and secondary HLH was diagnosed.The underlying primary diseases were lymphoma, hepatitis-associated aplastic anemia, Langerhans cell histiocytosis, systemic autoinflammatory disease and hereditary metabolic disease.Except for controlling HLH activation, the treatment of primary disease was also of great significance.Conclusions:Pediatric HLH is a group of syndrome with complex underlying diseases.Therefore, pediatricians should pay more attention to the stratified diagnosis of underlying primary diseases of pediatric secondary HLH.Related laboratory tests should be performed as soon as possible to determine the etiology after HLH is diagnosed.