单中心组织细胞坏死性淋巴结炎的临床特征性分析

Analysis of the Clinical Characteristics of Histiocytic Necrotizing Lymphadenitis in a Single Center

摘要: 目的：回顾性分析组织细胞坏死性淋巴结炎(HNL)患者的临床特征，比较儿童组和成人组的差异，以拓展对本病的认识及诊断，减少漏诊、误诊率。方法：收集2010年1月至2024年6月年在空军军医大学第一附属医院住院的82例HNL患者的临床特征，包括一般资料、临床表现、辅助检查(包括全身炎症指标NLR、PLR、LMR、SII和SIRI)、淋巴结病理活检、治疗及预后，并比较分析儿童组(<18岁)和成人组(≥18岁)患者临床特征的差异。结果：HNL患者共82例，男女比例为1.41:1。所有患者均会先后出现发热和淋巴结肿大，以双侧颈部淋巴结肿大(79.3%)为主，68.3%伴淋巴结肿痛，伴随症状：最常见扁桃体肿大(63.4%)、咳嗽(41.5%)，其次咽痛(26.8%)、头痛(22.0%)、脾大(20.7%)、皮疹(18.3%)、腹痛(15.9%)、肌肉关节疼痛(12.2%)，少数(8.5%)有口腔溃疡、鹅口疮、胸闷等症状。实验室检查：最常见LDH升高(93.9%)、ESR升高(81.7%)、PCT、IL-6升高(82.7%)、白细胞减少(78.0%)、铁蛋白升高(71.4%)，其次超敏CRP升高(66.7%)、白蛋白减少(65.9%)、中性粒细胞减少(53.7%)、血小板减少(50.0%)，少数肝功能异常(19.5%)，贫血少见(2.4%)。病原学：EBV、肺炎支原体、风疹病毒、HSV-1、CMV、链球菌的感染率分别为90.4%、43.6%、40.0%、30.0%、22.5%、21.9%，仅5.0%流感病毒感染，4.8%结核分支杆菌感染。免疫组化：组织细胞标志物CD68+、CD163+和MPO+分别为100%、84%和98%；浆细胞样树突状细胞标记物CD123+ 89%，CD3+、CD4+和CD8+ T细胞亚群分别占95%、85%和89%。72.0%的患者糖皮质激素治疗后体温恢复正常，随访10例(12.2%)患者复发；4例确诊前分别合并SLE、多发性大动脉炎、成人Still病、急性淋巴细胞白血病；2例3年后继发SLE和强直性脊柱炎。儿童组与成人组在性别(P = 0.001)、颈外淋巴结肿大(P = 0.021)、最大淋巴结直径(P = 0.049)、扁桃体肿大(P = 0.001)、肌肉关节疼痛(P = 0.023)、白蛋白减少(P = 0.01)、N (P = 0.042)、L (P < 0.001)、RBC (P = 0.044)的差异存在统计学意义，在全身炎症指标NLR (P < 0.001)、PLR (P < 0.001)、LMR (P = 0.002)、SIRI (P < 0.001)、SII (P < 0.001)均存在统计学差异。结论：HNL多见于男性儿童和年轻女性，临床表现多样且缺乏特异性，感染源众多，是否与本病相关还需深入研究，糖皮质激素治疗有效，有复发倾向，可与SLE先后发生。儿童患者更易发生扁桃体肿大、白蛋白减少、中性粒细胞减少，成人患者更易发生颈外淋巴结肿大、肌肉关节疼痛。儿童组与成人组全身炎症指标均存在统计学差异，而对远期生存质量的影响仍有待深入研究。

Abstract: Objective: To retrospectively analyze the clinical characteristics of patients with histiocytic necrotizing lymphadenitis (HNL), compare the differences between the pediatric group and the adult group, and expand the understanding and diagnosis of this disease to reduce the rate of missed diagnosis and misdiagnosis. Methods: The clinical characteristics of 82 patients with HNL who were hospitalized at the First Affiliated Hospital of Air Force Military Medical University from January 2010 to June 2024 were collected, including general information, clinical manifestations, auxiliary examinations (including systemic inflammatory indicators NLR, PLR, LMR, SII and SIRI), lymph node pathological biopsy, treatment and prognosis. The differences in clinical characteristics between the pediatric group (<18 years old) and the adult group (≥18 years old) were compared and analyzed. Results: A total of 82 patients with HNL were included, with a male-to-female ratio of 1.41:1. All patients presented with fever and lymphadenopathy successively, with bilateral cervical lymphadenopathy (79.3%) being the most common, and 68.3% accompanied by lymphadenopathy pain. The most common accompanying symptoms were tonsillar hypertrophy (63.4%) and cough (41.5%), followed by sore throat (26.8%), headache (22.0%), splenomegaly (20.7%), rash (18.3%), abdominal pain (15.9%), and muscle and joint pain (12.2%). A few patients (8.5%) had symptoms such as oral ulcers, thrush, and chest tightness. Laboratory tests: The most common were elevated LDH (93.9%), elevated ESR (81.7%), elevated PCT and IL-6 (82.7%), leukopenia (78.0%), and elevated ferritin (71.4%). Other common findings included elevated hypersensitive CRP (66.7%), decreased albumin (65.9%), decreased neutrophils (53.7%), and thrombocytopenia (50.0%). A few had abnormal liver function (19.5%), and anemia was rare (2.4%). Etiology: The infection rates of EBV, Mycoplasma pneumoniae, rubella virus, HSV-1, CMV, and Streptococcus were 90.4%, 43.6%, 40.0%, 30.0%, 22.5%, and 21.9%, respectively. Only 5.0% had influenza virus infection, and 4.8% had Mycobacterium tuberculosis infection. Immunohistochemistry: The expression rates of histiocyte markers CD68+, CD163+, and MPO+ were 100%, 84%, and 98%, respectively; the expression rate of plasmacytoid dendritic cell marker CD123+ was 89%, and the proportions of CD3+, CD4+, and CD8+ T cell subsets were 95%, 85%, and 89%, respectively. 72.0% of the patients had normal body temperature after glucocorticoid treatment. Follow-up of 10 patients (12.2%) showed recurrence. Four patients were diagnosed with SLE, polyarteritis nodosa, adult-onset Still’s disease, and acute lymphoblastic leukemia before the diagnosis of HNL. Two patients developed SLE and ankylosing spondylitis 3 years later. There were statistically significant differences between the pediatric group and the adult group in gender (P = 0.001), external cervical lymphadenopathy (P = 0.021), maximum lymph node diameter (P = 0.049), tonsillar hypertrophy (P = 0.001), muscle and joint pain (P = 0.023), decreased albumin (P = 0.01), N (P = 0.042), L (P < 0.001), and RBC (P = 0.044). There were also statistically significant differences in systemic inflammatory indicators NLR (P < 0.001), PLR (P < 0.001), LMR (P = 0.002), SIRI (P < 0.001), and SII (P < 0.001). Conclusion: HNL is more common in male children and young women, with diverse and non-specific clinical manifestations and multiple infection sources. Whether these sources are related to the disease requires further research. Glucocorticoid treatment is effective, but there is a tendency for recurrence, and it can occur before or after SLE. Children are more likely to have tonsillar hypertrophy, decreased albumin, and neutropenia, while adults are more prone to cervical lymphadenopathy and muscle and joint pain. There are statistically significant differences in systemic inflammatory indicators between the pediatric and adult groups, but the impact on long-term quality of life remains to be further studied.