Journal of Yeungnam Medical Science

영남대 의대 학술지 편집위원회 (Yeungnam University College of Medicine, Yeungnam University Institute Medical Science)
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Hemophagocytic lymphohistiocytosis with recurrent Kikuchi-Fujimoto disease

  • Lee, Sang Min (Department of Pediatrics, Yeungnam University Hospital) ;
  • Lim, Young Tae (Department of Pediatrics, Yeungnam University Hospital) ;
  • Jang, Kyung Mi (Department of Pediatrics, Yeungnam University College of Medicine) ;
  • Gu, Mi Jin (Department of Pathology, Yeungnam University College of Medicine) ;
  • Lee, Jong Ho (Department of Laboratory Medicine, Yeungnam University College of Medicine) ;
  • Lee, Jae Min (Department of Pediatrics, Yeungnam University College of Medicine)
  • 투고 : 2020.07.17
  • 심사 : 2020.09.14
  • 발행 : 2021.07.31
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초록

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limiting lymphadenitis. It is a benign disease mainly characterized by high fever, lymph node swelling, and leukopenia. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with clinical symptoms similar to those of KFD, but it requires a significantly more aggressive treatment. A 19-year-old Korean male patient was hospitalized for fever and cervical lymphadenopathy. Variable-sized lymph node enlargements with slightly necrotic lesions were detected on computed tomography. Biopsy specimen from a cervical lymph node showed necrotizing lymphadenitis with HLH. Bone marrow aspiration showed hemophagocytic histiocytosis. The clinical symptoms and the results of the laboratory test and bone marrow aspiration met the diagnostic criteria for HLH. The patient was diagnosed with macrophage activation syndrome-HLH, a secondary HLH associated with KFD. He was treated with dexamethasone (10 mg/m2/day) without immunosuppressive therapy or etoposide-based chemotherapy. The fever disappeared within a day, and other symptoms such as lymphadenopathy, ascites, and pleural effusion improved. Dexamethasone was reduced from day 2 of hospitalization and was tapered over 8 weeks. The patient was discharged on day 6 with continuation of dexamethasone. The patient had no recurrence at the 18-month follow-up.

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과제정보

This work was supported by the 2019 Yeungnam University Research Grant (219A480007).

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