Yoo In Hyuk, Na Hyunju, Bae E Young, Han Seung Beom, Lee Soo Young, Jeong Dae Chul, Kang Jin Han
Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea,
Eur J Pediatr. 2014 Sep;173(9):1193-9. doi: 10.1007/s00431-014-2306-6. Epub 2014 Apr 9.
Kikuchi-Fujimoto disease (KFD) is characterized by self-limiting regional lymphadenopathy with prolonged fever. Although the reported recurrence rate of KFD is known to be 3-4 %, this rate appears to be higher in our clinical experience, and rates up to 38.5 % have been previously reported. In this retrospective study, we reviewed medical records of children with pathologically confirmed KFD to investigate the factors associated with recurrent KFD. Enrolled children were divided into two groups according to the recurrence of KFD, and clinical and laboratory factors were compared between the two groups. The recurrence of KFD was determined based not on repeated pathologic confirmation but on the presence of clinical febrile lymphadenopathy. A total of 33 children with KFD, 26 boys (78.8 %) and 7 girls (21.2 %), with a median age of 12 years (9 months to 19 years), were enrolled. Thirty-one children (93.9 %) complained of fever, and most of the children (90.9 %) complained of cervical lymphadenopathy. Neutropenia (<1,500/μL) or lymphopenia (<1,500/μL) was observed in 51.5 %. Lactate dehydrogenase level, erythrocyte sedimentation rate, and C-reactive protein level were elevated in 90.9, 96.9, and 54.5 % of children, respectively. Fourteen children (42.4 %) experienced recurrent KFD, including ten children after biopsy and four children before and after biopsy. In a multivariate analysis, a past history of other systemic illnesses (p = 0.013) and a higher absolute lymphocyte count (p = 0.023) were significantly associated with recurrent KFD. These systemic illnesses were chronic idiopathic thrombocytopenic purpura, autoimmune thyroiditis, nephrotic syndrome, perinatal cytomegalovirus infection, and hemophagocytic lymphohistiocytosis.
Our results suggest that recurrent KFD is more frequent than reported, and recurrent KFD should be considered in children with a history of other systemic illnesses such as immune disorders.
菊池-藤本病（KFD）的特征是伴有长期发热的自限性局部淋巴结病。虽然已知KFD报告的复发率为3%-4%，但根据我们的临床经验，这一比率似乎更高，之前曾有高达38.5%的复发率报告。在这项回顾性研究中，我们回顾了经病理确诊为KFD的儿童的病历，以调查与KFD复发相关的因素。根据KFD的复发情况将入选儿童分为两组，并比较两组之间的临床和实验室因素。KFD的复发不是基于重复的病理确诊，而是基于临床发热性淋巴结病的存在来确定。共有33例KFD患儿入选，其中26例男孩（78.8%），7例女孩（21.2%），中位年龄为12岁（9个月至19岁）。31例患儿（93.9%）有发热症状，大多数患儿（90.9%）有颈部淋巴结病。51.5%的患儿出现中性粒细胞减少（<1500/μL）或淋巴细胞减少（<1500/μL）。乳酸脱氢酶水平、红细胞沉降率和C反应蛋白水平分别在90.9%、96.9%和54.5%的患儿中升高。14例患儿（42.4%）经历了KFD复发，其中10例在活检后复发，4例在活检前后复发。在多变量分析中，其他全身性疾病的既往史（p = 0.013）和较高的绝对淋巴细胞计数（p = 0.023）与KFD复发显著相关。这些全身性疾病包括慢性特发性血小板减少性紫癜、自身免疫性甲状腺炎、肾病综合征、围产期巨细胞病毒感染和噬血细胞性淋巴组织细胞增生症。
我们的结果表明，KFD复发比报告的更为频繁，对于有其他全身性疾病如免疫紊乱病史的儿童应考虑KFD复发。
