Dokić Milomir, Begović Vesna, Bojić Ivanko, Tasić Olga, Stamatović Dragana
Vojnomedicinska akademija, Klinika za infektivne i tropske bolesti, Beograd.
Vojnosanit Pregl. 2003 Sep-Oct;60(5):625-30. doi: 10.2298/vsp0305625d.
Kikuchi-Fujimoto disease (KFD), also know as histiocytic necrotizing lymphadenitis, is a benign disorder characterized histologically by necrotic foci surrounded by histiocytic aggregates, and with the absence of neutrophils. KFD was recognized in Japan, where it was first described in 1972. The disease is most commonly affecting young women. The cause of the disease is unknown, and its exact pathogenesis has not yet been clarified. Many investigators have postulated viral etiology of KFD, connecting it with Epstein Barr virus, human herpes simplex virus 6 parvo B 19, but also with toxoplasmic infection. Kikuchi-Fujimoto disease is usually manifested with lymphadenopathy and high fever, and is associated with lymphopenia, splenomegaly, and hepatomegaly with abnormal liver function tests, arthralgia, and weight loss. The disease has the tendency of spontaneous remission, with mean duration of three months. Single recurrent episodes of KFD have been reported with many years' pauses between the episodes. Kikuchi-Fujimoto disease may reflect systemic lupus erythematosus (SLE), and self-limited SLE-like conditions. Final diagnosis could only be established on the basis of typical morphological changes in the lymph node, and lymph node biopsy is needed for establishing the diagnosis. Lymphadenopathy in a patient with fever of the unknown origin could provide a clue to the diagnosis of lymphoma, tuberculosis, metastatic carcinoma, toxoplasmosis and infectious mononucleosis. As KFD does not have any classical clinical features and laboratory characteristics, it may lead to diagnostic confusion and erroneous treatment. We described a case of KFD, and suggested that this disease should be considered as a possible cause of fever of the unknown origin with lymphadenopathy.
菊池-藤本病(KFD),也称为组织细胞坏死性淋巴结炎,是一种良性疾病,其组织学特征为坏死灶被组织细胞聚集物包围,且无中性粒细胞。KFD在日本被首次认识,并于1972年首次被描述。该疾病最常影响年轻女性。病因不明,其确切发病机制尚未阐明。许多研究者推测KFD的病毒病因,将其与爱泼斯坦-巴尔病毒、人类疱疹病毒6型、细小病毒B19相关联,也与弓形虫感染有关。菊池-藤本病通常表现为淋巴结病和高热,并伴有淋巴细胞减少、脾肿大、肝功能检查异常的肝肿大、关节痛和体重减轻。该疾病有自发缓解的倾向,平均病程为三个月。曾有KFD单次复发的报道,发作之间间隔数年。菊池-藤本病可能反映系统性红斑狼疮(SLE)以及自限性SLE样病症。最终诊断只能基于淋巴结典型的形态学改变来确立,且需要进行淋巴结活检以明确诊断。不明原因发热患者的淋巴结病可能为淋巴瘤、结核病、转移性癌、弓形虫病和传染性单核细胞增多症的诊断提供线索。由于KFD没有任何典型的临床特征和实验室特点,可能导致诊断混淆和错误治疗。我们描述了一例KFD病例,并建议应将该疾病视为不明原因发热伴淋巴结病的可能病因。