Pepe F, Disma S, Teodoro C, Pepe P, Magro G
Ospedale Santo Bambino, UOC Ostetricia e Ginecologia e Pronto Soccorso, Catania, Italy.
Ospedale Cannizzaro, UOC Urologia, Catania, Italy.
Pathologica. 2016 Sep;108(3):120-129.
Kikuchi-Fujmoto disease (KFD), also known as "histiocytic necrotizing lymphadenitis", is a rare lymphadenitis of unknown origin, but with an excellent prognosis. It is more common in Asia, but isolated cases are also reported in America, Africa and Europe. The disease can have an acute or subacute course, usually develops in 1 to 3 weeks, with spontaneous resolution in 1-4 months. The main clinical sign is cervical lymphadenopathy, especially in the posterior cervical triangle with bulky and painful lymph nodes, usually affecting only one side; rare cases of generalized lymphadenopathy can be seen. This common clinical presentation can also be accompanied by nausea, vomiting, weight loss, weakness, headache and arthralgia. An extranodal extension of the disease, including involvement of skin, eye, and bone marrow localizations, has been rarely described. Most patients have leukopenia or neutropenia with a relative leukocytosis. At an ultrasound exploration of the affected lymph nodes, a hypoechoic aspect can be seen, with an external, thick and irregular hyperechoic ring. As there are no specific tests for KFD, the final diagnosis is histologically-based from lymph node excisional biopsy. Histological examination shows paracortical foci of coagulative necrosis containing karyorrhectic debris, which are surrounded by numerous CD68+/myeloperoxidase (MPO)+ histiocytes, CD68+/CD123+ plasmacytoid dendritic cells, and a minority of small- to large-sized CD8+lymphocytes and immunoblasts. Differential diagnosis mainly includes systemic lupus erithematous (SLE)-related lymphadenopathy and large cell lymphoma. The histological absence of neutrophils, plasmacells, as well as hematoxylin bodies, is a feature which argues against the diagnosis of SLE. In addition, the absence of auto-antibodies and anti-nuclear antibodies is useful in ruling out an autoimmune disorder. Early diagnosis of KFD is crucial to prevent the patients undergo extensive investigations related to suspected malignant lymphomas or other diseases.
菊池-藤本病（KFD），也被称为“组织细胞坏死性淋巴结炎”，是一种病因不明但预后良好的罕见淋巴结炎。它在亚洲更为常见，但美洲、非洲和欧洲也有散发病例报道。该病可呈急性或亚急性病程，通常在1至3周内发病，1至4个月内自行缓解。主要临床体征为颈部淋巴结病，尤其是颈后三角区出现肿大且疼痛的淋巴结，通常仅累及一侧；罕见有全身淋巴结病的病例。这种常见的临床表现还可能伴有恶心、呕吐、体重减轻、乏力、头痛和关节痛。该病的结外扩展，包括皮肤、眼睛和骨髓受累，很少被描述。大多数患者有白细胞减少或中性粒细胞减少伴相对淋巴细胞增多。在对受累淋巴结进行超声检查时，可看到低回声表现，周围有一个外部增厚且不规则的高回声环。由于菊池-藤本病没有特异性检查，最终诊断基于淋巴结切除活检的组织学结果。组织学检查显示副皮质区有凝固性坏死灶，内含核碎裂碎片，周围有大量CD68+/髓过氧化物酶（MPO）+组织细胞、CD68+/CD123+浆细胞样树突状细胞，以及少数小至大的CD8+淋巴细胞和免疫母细胞。鉴别诊断主要包括系统性红斑狼疮（SLE）相关淋巴结病和大细胞淋巴瘤。组织学上无中性粒细胞、浆细胞以及苏木精小体是反对SLE诊断的一个特征。此外，无自身抗体和抗核抗体有助于排除自身免疫性疾病。菊池-藤本病的早期诊断对于防止患者接受与疑似恶性淋巴瘤或其他疾病相关的广泛检查至关重要。
