Hemophagocytic Lymphohistiocytosis Associated with Histiocytic Necrotizing Lymphadenitis: A Clinical Study of 13 Children and Literature Review

Ying Yang, Hongyun Lian, Honghao Ma, Yunze Zhao, Qing Zhang, Li Zhang, Tianyou Wang, Zhigang Li, Rui Zhang
Journal of Pediatrics 2020 September 18

OBJECTIVE: To analyze the clinical characteristics and prognosis of pediatric hemophagocytic lymphohistiocytosis associated with histiocytic necrotizing lymphadenitis (HNL-HNL).

STUDY DESIGN: We retrospectively collected the clinical data of all HNL-HLH children enrolled in Beijing Children's Hospital from 2007 to 2019. The control patients with Epstein-Barr virus-associated HLH (EBV-HLH) and simple HNL (non-associated with HLH) were case-matched (1:2). The clinical features and prognosis were analyzed by case-control study. Cases of HNL-HLH in the literature were reviewed.

RESULTS: The male-to-female ratio of the 13 patients in our center was 9:4. The mean age of the patients at disease onset was 8.1 years (SD ±1.2), younger than that of the 16 patients in the literature (P = .017). Clinical presentations, especially rash and splenomegaly, and laboratory examination of HNL-HLH group were statistically different from EBV-HLH group, simple HNL group, and patients reported in the literature (P<0.05). Three patients were treated with immunosuppressive drugs or chemotherapy due to poor control of HLH. One patient died, and all 12 remaining patients survived, 2 of which developed autoimmune diseases. Kaplan-Meier survival curves showed no statistical difference among the three groups (P>0.05).

CONCLUSIONS: HNL-HLH is more common in school- and preschool-age children. Most patients have a favorable prognosis. Some patients suffer from relapses or develop autoimmune diseases. Prolonged follow-up should be carried out for patients with HNL-HLH.

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