Fei Xiuwen, Liu Si, Wang Bo, Dong Aimei
Department of Emergency, Peking University First Hospital, Beijing 100034, China.
Department of General Practice, Peking University First Hospital, Beijing 100034, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2024 Jun 18;56(3):533-540. doi: 10.19723/j.issn.1671-167X.2024.03.022.
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.
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.
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 ( < 0.05).
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.
分析组织细胞坏死性淋巴结炎(HNL)的临床资料,比较儿童与成人患者的异同,以加深临床医师对该疾病的认识,提高诊断率,减少误诊和误治。
收集2010年1月至2023年8月北京大学第一医院经活检确诊的组织细胞坏死性淋巴结炎住院患者的临床资料,分析其临床特征、实验室检查、病理特征、抗生素及糖皮质激素治疗情况及预后。按年龄分组,比较儿童组(<16岁)与成人组(≥16岁)临床特征、实验室检查、治疗及预后的差异。
81例纳入患者中,男42例,女39例。年龄中位数为21(14,29)岁,病程中位数为20.0(13.0,30.0)天,住院时间中位数为13.0(10.0,15.0)天。首发症状为发热、淋巴结肿大及二者兼有。所有患者均有不同部位、不同大小的淋巴结肿大,96.3%(81例中的78例)患者有颈部淋巴结肿大,50.6%(81例中的41例)有双侧颈部淋巴结肿大,55.6%(81例中的45例)有锁骨上、腋窝或腹股沟淋巴结肿大,淋巴结直径中位数为20.0(20.0,30.0)mm。仅1例患者无发热,其余80例患者有发热,体温峰值中位数为39.0(38.0,39.8)℃。伴随症状有皮疹(8.6%,81例中的7例)、乏力(34.6%,81例中的28例)、盗汗(8.6%,81例中的7例)、寒战(25.3%,81例中的25例)、肌肉酸痛(13.6%,81例中的11例)及关节痛(6.2%,81例中的5例)。有17例(21.0%)患者肝脾肿大,其中脾肿大12例(70.6%)。68.8%(80例中的55例)患者白细胞(WBC)计数降低,47.5%(80例中的38例)淋巴细胞(LY)比例升高,53.4%(73例中的39例)高敏C反应蛋白(CRP)升高,79.2%(72例中的57例)红细胞沉降率(ESR)升高,22.2%(81例中的18例)丙氨酸转氨酶(ALT)升高,27.2%(81例中的22例)天冬氨酸转氨酶(AST)升高,81.6%(76例中的62例)乳酸脱氢酶(LDH)升高。81例患者均行淋巴结活检,77.8%(81例中的63例)患者淋巴结大部分结构破坏或消失,16.0%(81例中的13例)淋巴结结构尚存,增生及正常淋巴结各占1.2%(81例中的1例),3.7%(81例中的3例)淋巴结结构正常。67例行免疫组化染色,CD3及CD68(KP1)阳性率分别为97.0%(67例中的65例),MPO阳性率为94.0%(67例中的63例)。本研究中,51例(63.0%)患者确诊后接受糖皮质激素治疗,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)天,糖皮质激素治疗效果最佳时体温可于当日降至正常。成人组与儿童组在住院时间、诱发因素、寒战、高敏CRP升高率、抗生素及糖皮质激素治疗方面差异有统计学意义(P<0.05)。
临床工作中,若遇到以不明原因发热、浅表淋巴结肿大、白细胞降低为临床特征,且普通抗生素治疗无效的病例,应考虑组织细胞坏死性淋巴结炎。应尽早行淋巴结活检以明确诊断,减少误诊和误治,治疗以对症治疗为主。糖皮质激素治疗有确切疗效。