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논문 기본 정보

자료유형
학술저널
저자정보
Sang Min Lee (Department of Pediatrics Yeungnam University Hospital Daegu Korea) Young Tae Lim (Department of Pediatrics Yeungnam University Hospital Daegu Korea) Kyung Mi Jang (Department of Pediatrics Yeungnam University College of Medicine Daegu Korea) Mi Jin Gu (Department of Pathology Yeungnam University College of Medicine Daegu Korea) Jong Ho Lee (Department of Laboratory Medicine Yeungnam University College of Medicine Daegu Korea) Jae Min Lee (Department of Laboratory Medicine Yeungnam University College of Medicine Daegu Korea)
저널정보
영남대학교 의과대학 Journal of Yeungnam Medical Science Yeungnam University Journal of Medicine 제38권 제3호
발행연도
2021.1
수록면
245 - 250 (6page)

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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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