Kikuchi's disease with hemophagocytic lymphohistiocytosis: A case report and literature review

INTRODUCTION: Kikuchi's disease (KD) is a rare form of necrotizing lymphadenitis that rarely occurs in association with hemophagocytic lymphohistiocytosis (HLH) in children. PATIENT CONCERNS: We report the case of a 4-year-5-month-old boy who suffered from fever, cervical lymphadenopathy, pancytopenia, hypertriglyceridemia, splenomegaly, low NK cell activity. DIAGNOSES: A diagnosis of KD with HLH was made based on the results of biopsy of cervical lymph node and HLH-2004 trial guidelines. INTERVENTIONS: The patient was treated with corticosteroids, cyclosporine, etoposide, continuous hemodiafiltration (HDF), and plasma exchange (PE). OUTCOMES: He 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. CONCLUSION: HLH 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.