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ObjectivesTo establish a diagnostic tree analysis (DTA) model based on ultrasonography (US) findings and clinical characteristics for differential diagnosis of common causes of cervical lymphadenopathy in children.MethodsA total of 242 patients (131 boys, 111 girls; mean age, 11.2 ± 0.3 years; range, 1 month–18 years) with pathologically confirmed Kikuchi disease (n = 127), reactive hyperplasia (n = 64), lymphoma (n = 24), or suppurative lymphadenitis (n = 27) who underwent neck US were included. US images were retrospectively reviewed to assess lymph node (LN) characteristics, and clinical information was collected from patient records. DTA models were created using a classification and regression tree algorithm on the basis of US imaging and clinical findings. The patients were randomly divided into training (70%, 170/242) and validation (30%, 72/242) datasets to assess the diagnostic performance of the DTA models.ResultsIn the DTA model based on all predictors, perinodal fat hyperechogenicity, LN echogenicity, and short diameter of the largest LN were significant predictors for differential diagnosis of cervical lymphadenopathy (overall accuracy, 85.3% and 83.3% in the training and validation datasets). In the model based on categorical parameters alone, perinodal fat hyperechogenicity, LN echogenicity, and loss of fatty hilum were significant predictors (overall accuracy, 84.7% and 86.1% in the training and validation datasets).ConclusionsPerinodal fat hyperechogenicity, heterogeneous echotexture, short diameter of the largest LN, and loss of fatty hilum were significant US findings in the DTA for differential diagnosis of cervical lymphadenopathy in children.Key Points• Diagnostic tree analysis model based on ultrasonography and clinical findings would be helpful in differential diagnosis of pediatric cervical lymphadenopathy.• Significant predictors were perinodal fat hyperechogenicity, heterogeneous echotexture, short diameter of the largest LN, and loss of fatty hilum.

Kikuchi-Fujimoto disease (KFD) is a reactive lymphadenitis of unclear etiology. To understand the pathogenesis of KFD, we performed targeted RNA sequencing of a well-characterized cohort of 15 KFD specimens with 9 non-KFD lymphadenitis controls. Two thousand and three autoimmunity-related genes were evaluated from archived formalin-fixed paraffin-embedded lymph node tissue and analyzed by a bioinformatics approach. Differential expression analysis of KFD cases compared to controls revealed 44 significantly upregulated genes in KFD. Sixty-eight percent of these genes were associated with the type I interferon (IFN) response pathway. Key component of the pathway including nucleic acid sensors, IFN regulatory factors, IFN-induced antiviral proteins, IFN transcription factors, IFN-stimulated genes, and IFN-induced cytokines were significantly upregulated. Unbiased gene expression pathway analysis revealed enrichment of IFN signaling and antiviral pathways in KFD. Protein–protein interaction analysis and a molecular complex detection algorithm identified a densely interacting 15-gene module of type I IFN pathway genes. Apoptosis regulator IFI6 was identified as a key seed gene. Transcription factor target analysis identified enrichment of IFN-response elements and IFN-response factors. T-cell-associated genes were upregulated while myeloid and B-cell-associated genes were downregulated in KFD. CD123+ plasmacytoid dendritic cells (PDCs) and activated T cells were noted in KFD. In conclusion, KFD is mediated by an aberrant type I interferon response that is likely driven by PDCs and T cells.

Differentiation between lymphadenopathy in potentially life-threatening systemic lupus erythematosus (SLE) and self-limiting necrotizing lymphadenitis, also called Kikuchi— Fujimoto disease (KFD), is difficult. In the past, co-occurrence of SLE and KFD has been described repeatedly in case reports. Here, we report a case of necrotizing lymphadenitis, describe the clinical and histopathologic features in detail and discuss the current literature. KFD may in fact be a histopathologic characteristic of SLE supporting the hypothesis that KFD is a rare manifestation of SLE. To clarify whether KFD is the same entity as lupus lymphadenitis, more cases with SLE and lymphadenopathy should be examined in detail. Lupus (2010) 19, 89—92.

Kikuchi-Fujimoto disease is a benign and self-limited disorder. The common clinical features are fever and cervical lymphadenitis. A 9-year-old girl with fever and cervical lymphadenitis was admitted because of persistent symptoms. A cervical lymph node biopsy showed the characteristic features of Kikuchi-Fujimoto disease. Herein, we will discuss the clinical features, diagnosis, and treatment of Kikuchi-Fujimoto disease and highlight the dramatic response when a patient was treated with hydroxychloroquine.

Kikuchi-Fujimoto disease (KFD) is a rare entity characterized by subacute necrotizing lymphadenopathy and frequently associated with fever. Young adults of Asian ancestry are most commonly affected, but it has been reported worldwide. Despite many studies in the literature, the cause of KFD remains uncertain. Histologically, KFD is characterized by paracortical lymph node expansion with patchy, well-circumscribed areas of necrosis showing abundant karyorrhectic nuclear debris and absence of neutrophils and eosinophils. Three evolving histologic patterns—proliferative, necrotizing, and xanthomatous—have been recognized. By immunohistochemistry, histiocytes in KFD are positive for myeloperoxidase. There is a marked predominance of T cells in the lesions (with mostly CD8-positive cells) with very few B cells. The differential diagnosis of KFD includes infectious lymphadenitis, autoimmune lymphadenopathy (primarily systemic lupus erythematosus), and lymphoma. Clinicians and pathologists are poorly familiar with this entity, which frequently causes significant diagnostic challenges.

Due to its nonspecific clinical characteristics, histiocytic necrotizing lymphadenitis (HNL) is often misdiagnosed as a suppurative cervical lymphadenitis and lymphoma. Thus, this study aimed to investigate the clinical characteristics of HNL in pediatric patients. We retrospectively identified 61 patients with histopathologically confirmed HNL. Clinical and laboratory data, including age, sex, clinical manifestations, laboratory investigations, histological discoveries, treatment, and outcomes, were collected from the medical records to determine associations with extracervical lymph node (LN) involvement. The mean age of patients was 9.7 ± 2.8 years (range, 1.5–14.0 years), and the male-to-female ratio was 2.2:1. The most common systemic symptom was fever in all patients. The median pre-admission and total durations of fever were 13.0 (interquartile range [IQR]: 9.0–22.5 days) and 22.0 days (IQR: 17.0–33.0 days), respectively. Patients with temporary fever (< 2 weeks) had a higher peak temperature and were more likely to undergo LN biopsy after admission than those with a prolonged fever (≥ 2 weeks). Multivariate analysis revealed that peak temperature ≥ 40 °C was significantly associated with a longer fever duration (P = 0.023). Laboratory values showed leukopenia (68.9%), which presented more frequently in solitary cervical LNs than in extracervical LNs (82.4% vs. 52.9%, p = 0.027) in patients with prolonged fever. Conclusions : HNL is often misdiagnosed in older children with persistent fever and lymphadenopathy, leading to unnecessary diagnostic tests and evaluations, inappropriate antibiotic administration, and mismanagement. A multidisciplinary team, including primary care providers, rheumatologists, and pathologists, can improve patient outcomes by increasing their awareness of this rare condition. What is Known: • Histiocytic necrotizing lymphadenitis (HNL) is characterized by fever, leukopenia, and neck lymphadenopathy with unknown etiology. • The lack of neutrophils or eosinophils in the histology, immunohistochemistry results help distinguish HNL from infectious causes.  Although HNL is a self-limiting disease, antibiotics and steroid treatments were used inappropriately. What is New: • A fever peak ≥ 40 °C was associated with a longer fever duration in HNL patients.  Leukopenia presented more frequently in solitary cervical lymph node (LNs) than in extracervical LNs in patients with prolonged fever. •  Steroids are not recommended as a routine treatment, however, in some severe or relapsing cases with  persistent symptoms, prednisolone (5 mg twice a day for 2 days) or other steroids (an equivalent dose of  prednisolone) responded favorably.

The lymphatic system is fundamentally important to cardiovascular disease, infection and immunity, cancer, and probably obesity--the four major challenges in healthcare in the 21st century. This Review will consider the manner in which new knowledge of lymphatic genes and molecular mechanisms has demonstrated that lymphatic dysfunction should no longer be considered a passive bystander in disease but rather an active player in many pathological processes and, therefore, a genuine target for future therapeutic developments. The specific roles of the lymphatic system in edema, genetic aspects of primary lymphedema, infection (cellulitis/erysipelas), Crohn's disease, obesity, cancer, and cancer-related lymphedema are highlighted.

Background Cervical suppurative lymphadenitis in children is commonly caused by Staphylococcus aureus or Streptococcus pyogenes. However, cases caused by Burkholderia multivorans (BM) are rare. The clinical presentation lacks specificity, making it difficult for clinicians to recognize, which may delay diagnosis and treatment. Case presentation We report a case of a 5-year-old boy admitted with recurrent fever and neck swelling. Initial treatment with meropenem and linezolid was ineffective, and symptoms persisted after 24 days of conservative therapy. Aspiration of pus yielded negative culture results. Definitive diagnosis was achieved through surgical biopsy of cervical lymph nodes, pathological examination, and metagenomic next-generation sequencing (mNGS), which identified BM as the causative pathogen. The patient was successfully treated with a combination of trimethoprim-sulfamethoxazole and meropenem. The cervical lesion exhibited granulomatous inflammation and was managed with adjunctive vacuum-assisted closure (VAC) therapy, resulting in complete wound healing without recurrence. Conclusions This study aims to raise awareness among all specialists about BM as a potential causative agent in cervical suppurative lymphadenitis. Early recognition and timely intervention can reduce misdiagnosis and missed diagnoses, improving patient outcomes.

Purpose To comprehensively analyze the clinical data of histiocytic necrotizing lymphadenitis (HNL) in adults with fever of unknown origin (FUO), with the aim of enabling precise diagnosis. Patients and methods A total of 15 HNL patients with FUO were enrolled. The analysis encompassed clinical manifestations, laboratory parameters 18 F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography ( 18 F-FDG PET/CT) imaging profiles, pathological features and therapeutic responses. Results All patients presented with fever and lymphadenopathy (predominantly cervical). Laboratory findings included leukopenia (3.28 × 10⁹/L [2.40–4.97]), elevated LDH (306 U/L [187–524]), ESR (40 mm/h [30–51]), ferritin (457.1 ng/mL [206-1823.3]), and CRP (25 mg/L [6.1–34.8]) 18 F-FDG PET/CT detected metabolic lymph node abnormalities in 13 cases, primarily cervical and axillary. The pathological features were extensive coagulative necrosis of lymph nodes with reactive hyperplasia of histiocytes as well as positive or scattered positivity IHC CD3, CD4, CD8 and CD68. Corticosteroid achieved favorable responses, with only 2 cases progressing during follow-up. Conclusion In clinical practice, patients with fever and lymphadenopathy should be given due attention. Pathological examination remains the gold standard for diagnosing HNL. Glucocorticoid therapy has proven effective, and the majority of patients with HNL exhibit a favorable prognosis.

This study aims to to establish a diagnosis model based on simple clinical features for children with cervical histiocytic necrotizing lymphadenitis or malignant lymphoma. Simple clinical features of pediatric patients were analyzed to develop a diagnosis model based on a comparison of classical machine-learning algorithms. This was a single-center retrospective study in a tertiary pediatrics hospital. Pediatric patients treated for cervical histiocytic necrotizing lymphadenitis or malignant lymphoma treated at our institution in recent 5 years were included. Demographic data and laboratory values were recorded and binary logistics regression analysis was applied to select possible predictors to develop diagnostic models with different algorithms. The diagnostic efficiency and stability of each algorithm were evaluated to select the best one to help establish the final model. Eighty-three children were included with 45 cases of histiocytic necrotizing lymphadenitis and 38 cases of malignant lymphoma. Peak temperature, white blood cell count, monocyte percentage, and urea value were selected as possible predictors based on the binary logistics regression analysis, together with imaging features already reported (size, boundary, and distribution of mass). In the ten-round random testing sets, the discriminant analysis algorithm achieved the best performance with an average accuracy of 89.0% (95% CI 86.2–93.6%) and an average AUC value of 0.971 (95% CI 0.957–0.995). Conclusion : A discriminant analysis model based on simple clinical features can be effective in differential diagnosis of cervical histiocytic necrotizing lymphadenitis and malignant lymphoma in children. Peak body temperature, white blood cell count, and short diameter of the largest mass are significant predictors. What is Known: • Several multivariate diagnostic models for HNL and ML have been proposed based on B-ultrasound or CT features in adults. • The differences between children and adults are nonnegligible in the clinical featues of HNL. What is New: • The study firstly report a large-sample diagnostic model between the HNL and MLin pediatric patients. • Non-imaging clinical features has also been proven with quite good diagnostic value.