Clinical features and prognosis of macrophage activation syndrome in children with histiocytic necrotizing lymphadenitis and literature review
10.3760/cma.j.issn.1673-4912.2024.12.009
- VernacularTitle:儿童组织细胞坏死性淋巴结炎合并巨噬细胞活化综合征临床特征和预后分析并文献复习
- Author:
Xue LI
1
;
Fan WU
1
;
Xiuli JU
1
Author Information
1. 山东大学齐鲁医院儿科，济南 250012
- Publication Type:Journal Article
- Keywords:
Histiocytic necrotizing lymphadenitis;
Macrophage activation syndrome;
Children
- From:
Chinese Pediatric Emergency Medicine
2024;31(12):924-928
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the clinical features and prognosis of macrophage activation syndrome（MAS）in children with histiocytic necrotizing lymphadenitis（HNL）.Methods:The clinical data of eight children with HNL combined with MAS admitted to the Department of Pediatrics,Qilu Hospital,Shandong University,from January 2021 to February 2023 were retrospectively analyzed for clinical symptoms,laboratory tests,pathological findings,treatments,and prognosis,and a literature review was performed.Results:Among the eigh patients，five were male and three were female,with an average age of onset of（10.91±4.07）years.All children presented with fever and lymph node enlargement，which were occasionally accompanied by symptoms such as rash and arthralgia.Laboratory tests mainly showed decreased blood cells，elevated ferritin，lactate dehydrogenase and liver enzymes，normal natural killer cell function，and increased inflammatory cytokines,particalarly interleukin-6,interleukin-10,interferon-γ,and tumor necrosis factor-α.All cases were pathologically diagnosed with HNL through lymph node biopsy.All children were treated with glucocorticoids combined with immunoglobulin and/or cyclosporine.Treatment outcomes indicated that one case relapsed，one progressed to Still's disease，while the remaining six patients survived.The literature review showed that a total of 32 cases of HNL complicated by MAS were reported over the past ten years，including 25 males and seven females，with an average age of（9.66±3.77）years old.All children initially presented with fever and lymph node enlargement，and laboratory tests showed that leukopenia and/or neutrophilia，along with increased lactate dehydrogenase，ferritin and liver enzymes.Hemophagocytosis in bone marrow was observed in 70.6% of the patients.Among the 30 children with documented medication records，19 patients showed imprevement following treatment with corticosteroids and intravenous inmunoglobulin，nine patients improved with corticosteroids combined with immunosuppressive therapy，one patient improved with symptomatic treatment alone，and one patient died.Conclusion:HNL in children can be combined with MAS.Most patients have a favorable prognosis.Elevated ferritin，lactate dehydrogenase and liver enzymes may be useful diagnostic markers for MAS in HNL.
