Histiocytic necrotizing lymphadenitis with central nervous system involvement in children: An analysis of clinical features.

OBJECTIVES

To summarize the clinical features of histiocytic necrotizing lymphadenitis (HNL) with central nervous system (CNS) involvement in children.

METHODOLOGY: A retrospective analysis was conducted on the clinical data of HNL-CNS children diagnosed and treated in Hebei Children's Hospital from January 2015 to January 2025.

RESULTS

A total of 22 HNL-CNS children were enrolled (17 boys, five girls; age 9.2 ± 3.5 years). Fever and lymphadenopathy occurred in all cases. Neurological manifestations included headache/vomiting (77.3%), convulsions (27.3%), and consciousness disorders (18.2%). Eight children presented with neurological symptoms before lymphadenopathy. Peripheral blood leukopenia was found in 68.2%. Cerebrospinal fluid (CSF) showed elevated pressure (81.8%), elevated white blood cell count (86.4%, mainly monocytes), and elevated protein (50.0%). Cranial MRI abnormalities were observed in 68.2%, predominantly in frontal/occipital lobes, with leptomeningeal enhancement in 13.6%. Anti-Mycoplasma pneumoniae antibody titers ≥1:160 were found in 50.0%. Antiviral therapy with acyclovir was given to 19 (86.4%) children, antibiotics were used in 17 (77.3%) children, intravenous corticosteroid (CS) therapy was provided to 19 (86.4%) children, and Cs combined with intravenous immunoglobulin (IVIG) was adopted in 8 (36.4%). Two out of three children who did not use Cs experienced recurrences.

CONCLUSION

HNL-CNS occurs predominantly in school-age boys. Neurological symptoms may precede lymphadenopathy. CSF findings are characterized by elevated pressure and mononuclear pleocytosis. MRI lesions commonly involve frontal and occipital lobes. Corticosteroid therapy yields favorable clinical outcomes and a low recurrence risk.