Have you ever encountered fever with swollen lymph nodes with a high misdiagnosis rate?

Source: Yimaitong

By: Love Peanuts

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A 7-year-old boy presented with “recurrent high fever with swollen cervical lymph nodes for 5 days”. 5 days ago, the child developed fever with no obvious incentive, with a fever peak of 39°C, and aversion to cold when hot. After oral administration of “ibuprofen”, the body temperature could be reduced to normal, and repeated at intervals of 4-6 hours, accompanied by swollen and tender cervical lymph nodes. , No cough and asthma, vomiting and diarrhea, the spirit is OK, the diet and sleep are OK, and the stool and urine are normal. Self-administration of “Cefdinir” for 2 days, the effect is not good.

Examination: Consciousness, good spirit, no rash all over the body, no chapped lips, negative strawberry tongue, palpable multiple swollen lymph nodes in the neck, about 3×2cm in size, Tough, with tenderness, range of motion, pharyngeal congestion, first-degree enlargement of bilateral tonsils, no pus emboli, no abnormality in cardiopulmonary and abdominal examination, and no swelling of extremities.

Laboratory examination: blood routine showed that the white blood cell count was 3.2×10^9/L, the neutrophil count was 1.0×10^9/L, the CRP was 28 mg/L, and the hemoglobin and platelet counts were normal.

Initial Diagnosis

School-age children, recurrent high fever with swollen cervical lymph nodes, physical examination of multiple swollen lymph nodes in the neck, accompanied by tenderness, blood routine examination showed leukopenia, elevated CRP, your preliminary What is the diagnosis? What is the treatment given?

Admission

The child was admitted to the hospital with “lymphadenitis”, complete blood biochemical examination showed elevated transaminases, no positive findings in etiological examination, ultrasound showed multiple enlarged lymph nodes in the neck, and echocardiography showed no abnormality , without coronary dilatation. Intravenous “cefmetazole” anti-infective treatment was given for 3 days, but the patient still had repeated high fever, and the cervical lymph nodes did not shrink. During this period, the child was in good spirits after the fever subsided, without conjunctival congestion, extremity peeling, red lips, chapped and other manifestations.

Diagnosed

Finally, with the consent of the parents, the cervical lymph node biopsy was completed, and the pathological result was diagnosed as histiocytic necrotizing lymphadenitis. Antibiotics were discontinued and glucocorticoid therapy was given, and the child was discharged from the hospital after normal body temperature.

Histiocytic necrotizing lymphadenitis(HNL) is an inflammatory disease of lymph nodes reported almost simultaneously by Japanese scholars Kikuchi and Fujimoto in 1972 , so the disease is also called Kikuchi disease or Kikuchi-Fujimoto disease (KFD). Due to the low incidence, complex and changeable clinical manifestations, and low awareness of the disease among some medical staff, the rate of misdiagnosis and treatment of the disease is very high. Some studies have found that the misdiagnosis rate can be as high as 100%, and the misdiagnosis rate is also as high as 90%.

However, HNL also has its own unique performance. Understanding and mastering the key points of HNL diagnosis and treatment can greatly reduce the misdiagnosis rate of patients and enable them to receive faster and more accurate treatment.

Quick questions and quick answers to help you quickly grasp the key points of HNL diagnosis and treatment

Question 1: What is the cause of HNL?

➤The etiology and pathogenesis of HNL remain unclear. At present, it is mostly considered to be an inflammatory immune reactive disease caused by pathogenic microorganisms such as viruses.

Question 2: What are the clinical manifestations of HNL?

➤HNL occurs in school-age children, fever, lymphadenopathy (most commonly Leukopenia and ineffective antibiotic therapy are the most characteristic clinical features.

➤In addition, there may also be rash, headache, gastrointestinal symptoms, respiratory symptoms, joint pain, elevated transaminases and CRP, and rapid erythrocyte sedimentation rate.

➤It should be noted that a small number of HNL children can involve the nervous system, and aseptic meningitis is the most common, which can be manifested as headache, nystagmus, and positive meningeal irritation signs.

Question 3: How is HNL diagnosed?

➤Pathological biopsy is the gold standard for HNL diagnosis. The typical pathological changes are: the normal structure of the lymph node disappears, extensive coagulation necrosis occurs, and there is reactive proliferation of surrounding histiocytes, phagocytosis, and no neutrophil infiltration; immunohistochemical examination shows CD8 +, CD4 +, and CD68 + infiltration as host.

➤CT examination showed speckled low-density necrosis in the central area of ​​the enlarged lymph nodes without calcification, which may have a certain prompting effect on the diagnosis.

Question 4: What treatments are available for HNL?

➤Currently, there is no unified treatment plan for HNL, and symptomatic treatment is the main clinical treatment.

➤Non-steroidal anti-inflammatory drugs (ibuprofen, acetaminophen) alone are effective for some children; some children no longer have body temperature after lymph node biopsy.

➤For patients with poor response to NSAIDs, glucocorticoids can be added after complete pathological biopsy, but there is currently no unified guideline for the specific dose and course of treatment. Intravenous dexamethasone 0.2-0.3 mg/(kg·d) or oral prednisolone 1-2 mg/(kg·d) are commonly used, and the dosage can be increased according to the specific condition of the child. Hormone application course of 2 weeks to 3 months.

➤For those with poor hormone effect, hydroxychloroquine can be added at a dose of 5-7mg/(kg·d).

➤Antibiotic treatment is not effective.

Question 5: What is the prognosis for HNL?

➤HNL is a benign, self-limiting disease with a course of 1-4 months. Most of the prognosis is good, but there are still about 3.3%-7.1% of children with recurrence, and a small number of children can have multiple recurrences. Some scholars believe that the recurrence of HNL may be related to the insufficient course of hormone application, so it is recommended to reduce the dose slowly in children who use hormones.

➤In addition, a small number of children may be accompanied by autoimmune diseases such as systemic lupus erythematosus and Hashimoto’s thyroiditis, and follow-up should be paid attention to.

References:

1. Li Fangfang, Zhao Linsheng, Li Chongwei. Clinical analysis of 70 children with histiocytic necrotizing lymphadenitis and the significance of lymph node biopsy[J].Chinese Journal of Practical Pediatrics,2021,36(17) :1325-1327.DOI: 10.3760/cma.j.cn101070-20200326-00514.

2. Shi Yunfeng, Wu Benquan, Chen Jianning, et al. Analysis of clinical features of histiocytic necrotizing lymphadenitis[J]. New Medicine, 2013(11):4.

3. Zhou Xiaojun, Zhang Qianzhong. A case of histiocytic necrotizing lymphadenitis recurrence after 6 years[J].Chinese Journal of Practical Pediatrics, 2011, 26(9):2.

4. Ma Haixing. Analysis of clinical and pathological features of histiocytic necrotizing lymphadenitis in children [D]. Ningxia Medical University, 2021. DOI: 10.27258/d.cnki.gnxyc.2021.000021.< /p>

5. Ke Yan, Feng Haifeng, Zheng Yi, Zhao Zhiwei, Jia Yanlong. Imaging manifestations and clinical analysis of histiocytic necrotizing lymphadenitis[J]. Journal of Medical Imaging, 2021, 31( 03):532-535.

6. Wu Yunshuang, Han Yang, Meng Fanrong, Yu Qun, Xu Jianteng, Xie Weizhen, Kai Shenfeng. Construction of a classification tree model for the risk of nervous system damage in patients with histiocytic necrotizing lymphadenitis[J] ].International Journal of Neurology and Neurosurgery,2022,49(01):41-45.DOI:10.16636/j.cnki.jinn.1673-2642.2022.01.009.

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