Department of Laboratories, 7274Seattle Children's Hospital, Seattle, WA, USA.
Department of Laboratory Medicine and Pathology, 7284University of Washington, Seattle, WA, USA.
Pediatr Dev Pathol. 2022 Sep-Oct;25(5):538-547. doi: 10.1177/10935266221103882. Epub 2022 May 18.
Kikuchi-Fujimoto disease (KFD) is a rare, benign, and self-limited disease that presents with cervical lymphadenopathy and systemic symptoms. Histologic evaluation is often necessary to differentiate KFD from other entities.
Electronic medical records and diagnostic material were reviewed for 14 children diagnosed with KFD and 6 children diagnosed with infectious mononucleosis (IM) from 2013-2021. Four cases of KFD were further characterized using targeted DNA-based next-generation sequencing.
Systemic symptoms were present in 86% (n = 12/14) of KFD patients, the most common being fever. Laboratory values worrisome for malignancy included cytopenia(s) (n = 9/12), elevated ESR and/or CRP (n = 9/12), elevated ferritin (n = 7/7), and elevated LDH (n = 7/10). Histologically, lymph nodes showed characteristic necrotic foci without neutrophils surrounded by MPO+ "crescentic" histiocytes. Immunoblasts and CD123+ plasmacytoid dendritic cells (pDCs) were also increased surrounding the necrosis. IM lymph nodes showed similar features when necrosis was present but increases in pDCs were patchy and rare neutrophils were seen in the necrotic foci. Molecular analysis of 4 KFD cases did not identify pathogenic variants.
While the signs/symptoms of KFD are worrisome, there are pathologic features that help differentiate it from potential mimics. We did not identify characteristic molecular features to aid in the work-up of these cases.
Kikuchi-Fujimoto 病（KFD）是一种罕见的良性、自限性疾病，表现为颈淋巴结病和全身症状。组织学评估对于将 KFD 与其他实体区分开来通常是必要的。
对 2013 年至 2021 年间诊断为 KFD 的 14 例儿童和诊断为传染性单核细胞增多症（IM）的 6 例儿童的电子病历和诊断材料进行了回顾性分析。对 4 例 KFD 病例进一步采用靶向 DNA 二代测序进行特征描述。
86%（12/14）的 KFD 患者存在全身症状，最常见的是发热。提示恶性肿瘤的实验室值包括血细胞减少症（n=9/12）、ESR 和/或 CRP 升高（n=9/12）、铁蛋白升高（n=7/7）和 LDH 升高（n=7/10）。组织学上，淋巴结显示特征性无中性粒细胞的坏死灶，周围有 MPO+“新月形”组织细胞。在坏死周围，免疫母细胞和 CD123+浆细胞样树突状细胞（pDC）也增加。当坏死存在时，IM 淋巴结显示出相似的特征，但 pDC 增加呈斑片状，坏死灶中罕见中性粒细胞。对 4 例 KFD 病例的分子分析未发现致病性变异。
虽然 KFD 的体征/症状令人担忧，但有一些病理特征有助于将其与潜在的类似物区分开来。我们没有发现有助于这些病例检查的特征分子特征。
