Famularo Giuseppe, Giustiniani Maria Cristina, Marasco Angela, Minisola Giovanni, Nicotra Giulio Cesare, De Simone Claudio
Department of Internal Medicine, San Camillo Hospital, Rome, Italy.
Am J Hematol. 2003 Sep;74(1):60-3. doi: 10.1002/ajh.10335.
We describe a young woman with two severe episodes of Kikuchi Fujimoto disease occurring 16 years apart. Both episodes were proven by biopsy, and on the second occasion the patient remained dependent on high-dose prednisone for more than 6 months in order to control inflammation and achieve a reduction in cervical lymph node size. The second lymph node biopsy showed leukocytoclastic vasculitis in addition to the typical features of Kikuchi Fujimoto disease, but, even though the clinical interpretation of this finding was unclear, we documented no clinical or laboratory evidence of the development of other serious systemic disease over 20 years of follow-up. Kikuchi Fujimoto disease is considered a disorder with a self-limited course and a favorable outcome. However, on the basis of our experience with this patient and data from peer-reviewed literature, we suggest that this generally accepted postulate should be revised.
我们描述了一位年轻女性，她相隔16年发生了两次严重的菊池富士本氏病发作。两次发作均经活检证实，第二次发作时，患者为控制炎症并使颈部淋巴结缩小，连续6个多月依赖大剂量泼尼松治疗。第二次淋巴结活检显示，除了菊池富士本氏病的典型特征外，还有白细胞破碎性血管炎。然而，尽管这一发现的临床意义尚不清楚，但在20多年的随访中，我们未发现其他严重全身性疾病发生的临床或实验室证据。菊池富士本氏病被认为是一种病程自限、预后良好的疾病。然而，基于我们对该患者的经验以及同行评审文献中的数据，我们建议应修订这一普遍接受的假设。
