The Ruth and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Semin Arthritis Rheum. 2010 Jun;39(6):515-20. doi: 10.1016/j.semarthrit.2009.03.003. Epub 2009 May 28.
Kikuchi Fujimoto disease (KFD) is a rare, benign disorder, usually characterized by cervical lymphadenopathy. Most available data on KFD has come from the Far East. We examined the characteristics of KFD in Israel.
A retrospective analysis of the records of all patients diagnosed as KFD in seven medical centers in Israel and all the cases previously reported as having occurred in Israel in the literature.
Nineteen patients were included, 13 new cases and six from the literature. Mean age of patients was 23 (range 9-50) years. Female/male ratio was 1.1:1. Cervical lymphadenopathy, the hallmark of KFD in the Far East (97%), was less frequent in Israel (44%). However, Israeli patients presented more often with generalized (26%) or retroperitoneal (21%) lymphadenopathy (P < 0.01). Systemic signs such as fever (73%), night sweats (21%), weight loss (21%), hepatomegaly or splenomegaly (25%), and elevated sedimentation rate (52%) were more common in Israeli patients compared to most reports from other parts of the world, excluding Germany (P < 0.05). Leukopenia was evident in most Israeli patients (72%) in contrast to other countries (P < 0.01). Clinical presentation of KFD in Germany was comparable to Israel in most aspects.
The clinical presentation of KFD in Israel often resembles a systemic disease with fever, leukopenia and generalized or retroperitoneal lymphadenopathy in more than half of the cases, contrary to the presentation in the Far East, which typically includes cervical lymphadenitis, and less frequently, systemic manifestations.
Kikuchi Fujimoto 病(KFD)是一种罕见的良性疾病,通常表现为颈部淋巴结病。大多数关于 KFD 的可用数据来自远东地区。我们研究了 KFD 在以色列的特征。
对以色列 7 家医疗中心诊断为 KFD 的所有患者的病历进行回顾性分析,并对文献中报道的此前在以色列发生的所有病例进行分析。
共纳入 19 例患者,其中 13 例为新发病例,6 例来自文献。患者平均年龄为 23 岁(范围 9-50 岁)。女性/男性比例为 1.1:1。远东地区 KFD 的标志性特征是颈部淋巴结病(97%),在以色列则较为少见(44%)。然而,以色列患者更常出现全身性(26%)或腹膜后(21%)淋巴结病(P < 0.01)。与世界其他地区(不包括德国)相比,以色列患者更常出现全身症状,如发热(73%)、盗汗(21%)、体重减轻(21%)、肝脾肿大(25%)和血沉升高(52%)(P < 0.05)。与其他国家相比,大多数以色列患者(72%)存在白细胞减少(P < 0.01)。德国的 KFD 临床表现与以色列在大多数方面相似。
以色列 KFD 的临床表现常类似于全身性疾病,超过一半的患者有发热、白细胞减少和全身性或腹膜后淋巴结病,与远东地区以颈部淋巴结炎为主要表现、较少出现全身表现的情况不同。