아급성 괴사성 임파선염의 임상양상과 비전형적인 증례 소개

Abstract

Background:Subacute necrotizing lymphadenitis (SNL) or Kikuchi-Fujimoto disease, first described in 1972, is a benign process of lymphatic system that consists of lymphadenopathy which may be accopanied by fever, rash and some hematologic alterations. It affects usually young people, mostly women. Even though this disease is self-limited, many cases are misidentified as malignant lymphoma. The purpose of this study is to report the clinical characteristics, many laboratory tests and pathologic finding and to elaborate the criteria that are useful in distinguishing from fever cause. We are going to present some rare cases and fatal cases. Methods:Authors evaluated 116 patients, who were diagnosed as Subacute necrotizing lymphadenitis on excisional biopsy with retrospective chart review at Yonsei Medical Center from January 1993 to May 2000. Results:The mean age was 26.4±10.9 years (range:5～63 years) and the male to female ratio was 1:2.28. The most common symptoms were cervical lymphadenopathy (89.7%) and fever (57.8%), and the duration of symptom was generally less than 8 weeks. Leukopenia (74.4%), relative lymphocytosis (33.3%) and DIC (7 cases) were hematologic abnormalities. Recently, we experienced patients showing atypical clinical findings which were pancytopenia, DIC (disseminated intravascular coagulopathy), hypotension or debilitating symptoms. Steroid pulse or high dose steroid and immunosuppressant therapy were performed in atypical cases. Sj?gren some cases were rarely associated with systemic diseases such as systemic lupus erythematosus (4 cases), Sj?gren disease (2 cases), rheumatoid arthritis (3 cases), aseptic meningitis (2 cases) or adult Still's disease (1 case). Thirteen cases (10.9%) were recurrent, and two cases died. Conclusion:Subacute necrotizing lymphadenitis should be considered in the differential diagnosis of fever cause in the patients which had localized cervical adenopathy, unresponsive to antibiotic therapy, especially in young women. We report atypical or fatal cases and need clinical attention to this kind of possibility.