A Case of Subacute Necrotizing Lymphadenitis Complicated by Hemophagocytic Syndrome

Abstract

A 29-year-old male was admitted to our hospital with persistent high fever. Examination revealed painful swollen cervical lymph nodes. Laboratory tests revealed bicytopenia, coagulopathy, and elevated serum lactate dehydrogenase and ferritin levels. Bone marrow aspiration revealed hemophagocytosis; therefore, the patient was diagnosed as having hemophagocytic syndrome (HPS). Findings of fine-needle aspiration biopsy suggested the diagnosis of malignant lymphoma. On day 6 after admission, progression of bicytopenia was observed. Laboratory examination revealed a white blood cell count of 1600/μl and platelet count of 5000/μl. Lymph node biopsy was performed to identify the cause of the HPS, and histopathological examination revealed features consistent with subacute necrotizing lymphadenitis (SNL), also known as Kikuchi-Fujimoto disease (KFD). Steroid pulse therapy was initiated for HPS on the same day as the lymph node biopsy. The patient’s clinical symptoms resolved within 2 weeks. Although the prognosis of KFD is generally good, with resolution within approximately 1–3 months with symptomatic treatment, KFD can occasionally be fatal. We encountered a rare case of KFD that was complicated by HPS. This case highlights the need to bear in mind the diagnosis of KFD as one of the causes of HPS and that lymph node biopsy should be rapidly performed in patients in poor general condition.