• Wei Duan, Zheng-Hui Xiao, Long-Gui Yang, and Hai-Yan Luo.
• Department of Pediatric Emergency Center, Hunan Children's Hospital, Changsha, Hunan,China.
• Medicine (Baltimore). 2020 Dec 18; 99 (51): e23500.

IntroductionKikuchi's disease (KD) is a rare form of necrotizing lymphadenitis that rarely occurs in association with hemophagocytic lymphohistiocytosis (HLH) in children.Patient ConcernsWe report the case of a 4-year-5-month-old boy who suffered from fever, cervical lymphadenopathy, pancytopenia, hypertriglyceridemia, splenomegaly, low NK cell activity.DiagnosesA diagnosis of KD with HLH was made based on the results of biopsy of cervical lymph node and HLH-2004 trial guidelines.InterventionsThe patient was treated with corticosteroids, cyclosporine, etoposide, continuous hemodiafiltration (HDF), and plasma exchange (PE).OutcomesHe showed a complete response to therapy, and his condition gradually improved. He was discharged on day 45 after admission due to his good recovery status.ConclusionHLH can be associated with KD, especially in childhood, and may have an aggressive clinical course. Continuous HDF and PE and chemotherapy should be reserved for those patients who fail to respond to IVIG and corticosteroids.Copyright © 2020 the Author(s). Published by Wolters Kluwer Health, Inc.

19 keywords...

hide…