成人及儿童组织坏死性淋巴结炎临床特征及治疗

Clinical characteristics and treatment in adults and children with histiocytic necroti-zing lymphadenitis

目的:分析组织坏死性淋巴结炎(histiocytic necrotizing lymphadenitis,HNL)的临床资料,比较成人及儿童临床资料的异同,加深临床医师对该病的认识,提高诊断率,减少误诊、误治。方法:收集北京大学第一医院2010年1月至2023年8月活检病理诊断为组织坏死性淋巴结炎住院患者的临床资料,分析组织坏死性淋巴结炎的临床特征、实验室检查、病理特征、抗生素及糖皮质激素治疗及转归情况。根据年龄进行分组,比较儿童组(<16岁)和成人组(≥16岁)患者上述临床资料的差异。结果:入组的81例患者中,男42例,女39例,中位年龄为21(14,29)岁,中位病程为20.0(13.0,30.0)d,中位住院时长为13.0(10.0,15.0)d。首发症状有发热、淋巴结肿大及发热和淋巴结肿大同时起病。所有患者均有不同部位、不同大小的淋巴结肿大,96.3%(78/81)的患者伴颈部淋巴结肿大,双侧颈部淋巴结都肿大者占50.6%(41/81),锁骨上、腋窝或腹股沟淋巴结肿大者占55.6%(45/81),中位淋巴结直径为20.0(20.0,30.0)mm。仅有1例患者不伴有发热,其余80例患者均有发热,中位峰值体温为39.0(38.0,39.8)℃。伴随症状:皮疹7例(8.6%,7/81)、乏力28例(34.6%,28/81)、盗汗7例(8.6%,7/81)、畏寒寒战25例(25.3%,25/81)、肌肉酸痛11例(13.6%,11/81)、关节痛5例(6.2%,5/81)。肝脾肿大有17例(21.0%,17/81),其中脾大12例,占肝脾大的70.6%(12/17)。68.8%(55/80)的患者白细胞(white blood cells,WBC)计数下降,47.5%(38/80)淋巴细胞(lymphocyte,LY)比例升高,53.4%(39/73)超敏C反应蛋白(C-reactive protein,CRP)升高,79.2%(57/72)红细胞沉降率(erythrocyte sedimentation rate,ESR)升高,22.2%(18/81)谷丙转氨酶(alanine transaminase,ALT)升高,27.2%(22/81)谷草转氨酶(aspartate transaminase,AST)升高,81.6%(62/76)乳酸脱氢酶(lactate dehydrogenase,LDH)升高。81例患者均进行了淋巴结活检病理检查,77.8%(63/81)的患者淋巴结活检病理组织学表现为淋巴结内结构大部分破坏或消失,16.0%(13/81)淋巴结结构尚存或存在,淋巴结结构增生、淋巴结结构正常各占1.2%(1/81),3.7%(3/81)的患者未记录淋巴结结构。67例进行了免疫组织化学染色,CD3+、CD68(KP1)+分别占97.0%(65/67),MPO+占94.0%(63/67)。51例(63.0%,51/81)患者在明确诊断后给予糖皮质激素治疗,其中糖皮质激素治疗后体温恢复正常的中位数时间为1.0(1.0,4.0)d,糖皮质激素治疗效果最好时体温可当天下降至正常。成人组和儿童组在住院时长、是否有前驱诱因、是否伴随畏寒寒战、超敏CRP升高率、是否应用抗生素、是否应用糖皮质激素治疗方面差异有统计学意义(P<0.05)。结论:临床上如出现不明原因发热、浅表淋巴结肿大及白细胞减少为临床特征的病例,且一般抗感染药物治疗无效时,应考虑组织坏死性淋巴结炎,需尽早行淋巴结活检以明确诊断,减少误诊、误治,治疗以对症治疗为主,糖皮质激素治疗疗效确切。

Objective:To analyze the clinical data of histiocytic necrotizing lymphadenitis(HNL),comparing the similarities and differences between children and adults,to deepen the understanding of the disease by clinical physicians,and to improve diagnostic rate and reduce misdiagnosis and mistreatment.Methods:The clinical data of hospitalized patients with histiocytic necrotizing lymphadenitis diagnosed by biopsy from January 2010 to August 2023 in Peking University First Hospital were collec-ted,and the clinical features,laboratory examinations,pathological features,treatments with antibiotics and glucocorticoids,and prognosis of histiocytic necrotic lymphadenitis were analyzed.Grouped based on age,the differences of clinical characteristics,laboratory tests,treatment,and prognosis between the children group(<16 years old)and the adult group(≥16 years old)were compared.Results:Among the 81 enrolled patients,there were 42 males and 39 females.The median age was 21(14,29)years,the median duration of disease was 20.0(13.0,30.0)days,and the median length of hospital stay was 13.0(10.0,15.0)days.The first symptoms were fever,lymphadenopathy,and both.All the patients had enlarged lymph nodes with different parts and sizes,96.3%(78 of 81)of the patients had cervical lymphadenopathy,50.6%(41 of 81)had bilateral cervical lymphadenopathy,55.6%(45 of 81)had supraclavicular,axillary or inguinal lymphadenopathy,and the median lymph node diameter was 20.0(20.0,30.0)mm.Only one patient had no fever,the other 80 patients had fever,the median peak body temperature was 39.0(38.0,39.8)℃.Accompanying symptoms:rash(8.6%,7/81),fatigue(34.6%,28/81),night sweating(8.6%,7/81),chills(25.3%,25/81),muscle soreness(13.6%,11/81),and joint pain(6.2%,5/81).There were 17 cases(21.0%,17/81)of hepatosplenomegaly,of which 12 cases(70.6%,12/17)were splenomegaly.68.8%(55/80)of patients had a decrease in white blood cell(WBC)count,with 47.5%(38/80)increased in lymphocyte(LY)proportion,53.4%(39/73)increased in high-sensitivity C-reactive protein(CRP),79.2%(57/72)increased in erythrocyte sedimentation rate(ESR),22.2%(18/81)increased in alanine transaminase(ALT),27.2%(22/81)elevated in aspartate transaminase(AST),and 81.6%(62/76)elevated in lactate dehydrogenase(LDH).All the 81 patients underwent lymph node biopsy,and 77.8%(63/81)of the patients showed that most of the structures in the lymph nodes were destroyed or disappeared,and 16.0%(13/81)of the lymph nodes were still in existence,hyperplasia and normal lymph node were 1.2%(1/81)respectively,and 3.7%(3/81)had normal lymph node structures.Immunohistochemical staining was performed in 67 cases.The percentages of CD3+and CD68(KP1)+were respectively 97.0%(65/67),and MPO+were 94.0%(63/67).In the study,51 patients(63.0%,51/81)were treated with glucocorticoid therapy after diagnosis.The median time for temperature to return to normal was 1.0(1.0,4.0)days after glucocorticoid therapy.when the glucocorticoid treatment worked best,the body temperature could drop to normal on the same day.There were significant differences in length of stay,predisposing factors,chills,the rate of increase in high-sensitivity CRP,antibiotic and glucocorticoid treatment between the adults and children groups(P<0.05).Conclusion:In clinical practice,if there are cases with unexplained fever,superficial lymph node enlargement,and reduced white blood cells as clinical characteristics,and general antibiotics treatment is ineffective,histiocytic necrotic lymphadenitis should be considered.Lymph node biopsy should be performed as early as possible to clarify the diagnosis,reduce misdiagnosis and mistreatment,and symptomatic treatment should be the main treatment.Glucocorticoids therapy has a definite therapeutic effect.