OBJECTIVE: To investigate the expressions of Notch1 and Hes1 in diffuse large B-cell lymphoma (DLBCL), and their correlations with clinical features. METHODS: Immunohistochemistry (IHC) was performed on DLBCL samples (54 cases) and lymphadenitis tissues (20 cases) to evaluate the expressions of Notch1 and Hes1, and analyze their correlations with clinical characteristics of patients. Based on Oncomine database, the expressions of Notch1 and Hes1 mRNA and DNA were also explored. RESULTS: IHC result showed that the positive expression rates of Notch1 and Hes1 in DLBCL patients were significantly higher than those in the control group (P <0.05). In DLBCL patients, the expression of Notch1 was closely associated with B symptoms, Ann Arbor stage, lymphocyte count and the level of lactate dehydrogenase (P <0.05), while the expression level of Hes1 was significantly higher in patients with B symptoms (P <0.05). Notch⁺/Hes1⁺ expression was found in 21 DLBCL tissues (38.9%), and there was a correlation between Notch1 and Hes1 expression (r =0.296, P <0.05). Bioinformatics analysis (Oncomine database) showed that the mRNA expressions of Notch1 and Hes1 in the Brune dataset were significantly higher than those in the control tissues (P <0.05). CONCLUSION The expressions of Notch1 and Hes1 in DLBCL are significantly higher than those in lymphadenitis, and correlated with B symptoms and Ann Arbor stage, suggesting that Notch1 and Hes1 play important roles in the occurrence and development of DLBCL.
Humans ; Cell Line ; Clinical Relevance ; Lymphadenitis ; Lymphoma, Large B-Cell, Diffuse/pathology* ; Prognosis ; RNA, Messenger

Objective: To investigate the value of plasma cells for diagnosing lymph node diseases. Methods: Common lymphadenopathy (except plasma cell neoplasms) diagnosed from September 2012 to August 2022 were selected from the pathological records of Changhai Hospital, Shanghai, China. Morphological and immunohistochemical features were analyzed to examine the infiltration pattern, clonality, and IgG and IgG4 expression of plasma cells in these lymphadenopathies, and to summarize the differential diagnoses of plasma cell infiltration in common lymphadenopathies. Results: A total of 236 cases of lymphadenopathies with various degrees of plasma cell infiltration were included in the study. There were 58 cases of Castleman's disease, 55 cases of IgG4-related lymphadenopathy, 14 cases of syphilitic lymphadenitis, 2 cases of rheumatoid lymphadenitis, 18 cases of Rosai-Dorfman disease, 23 cases of Kimura's disease, 13 cases of dermal lymphadenitis and 53 cases of angioimmunoblastic T-cell lymphoma (AITL). The main features of these lymphadenopathies were lymph node enlargement with various degrees of plasm cell infiltration. A panel of immunohistochemical antibodies were used to examine the distribution of plasma cells and the expression of IgG and IgG4. The presence of lymph node architecture could help determine benign and malignant lesions. The preliminary classification of these lymphadenopathies was based on the infiltration features of plasma cells. The evaluation of IgG and IgG4 as a routine means could exclude the lymph nodes involvement of IgG4-related dieases (IgG4-RD), and whether it was accompanied by autoimmune diseases or multiple-organ diseases, which were of critical evidence for the differential diagnosis. For common lesions of lymphadenopathies, such as Castleman's disease, Kimura's disease, Rosai-Dorfman's disease and dermal lymphadenitis, the expression ratio of IgG4/IgG (>40%) as detected using immunhistochemistry and serum IgG4 levels should be considered as a standard for the possibility of IgG4-RD. The differential diagnosis of multicentric Castleman's diseases and IgG4-RD should be also considered. Conclusions: Infiltration of plasma cells and IgG4-positive plasma cells may be detected in some types of lymphadenopathies and lymphomas in clinicopathological daily practice, but not all of them are related to IgG4-RD. It should be emphasized that the characteristics of plasma cell infiltration and the ratio of IgG4/IgG (>40%) should be considered for further differential diagnosis and avoiding misclassification of lymphadenopathies.
Humans ; Castleman Disease/pathology* ; Plasma Cells/pathology* ; Immunoglobulin G4-Related Disease ; China ; Lymphadenopathy/pathology* ; Inflammation/pathology* ; Lymph Nodes/pathology* ; Diagnosis, Differential ; Lymphadenitis/pathology* ; Immunoglobulin G/metabolism*

Objective: To explore the clinical features and prognosis of children with histiocytic necrotizing lymphadenitis (HNL). Methods: The clinical data of 118 children with HNL diagnosed and treated in the Department of Rheumatology and Immunology of Children's Hospital, Capital Institute of Pediatrics from January 2014 to December 2021 were retrospectively analyzed. The clinical symptoms, laboratory examination, imaging examination, pathological findings, treatment and follow-up were analyzed. Results: Among the 118 patients, 69 were males and 49 were females. The age of onset was 10.0 (8.0, 12.0) years, ranging from 1.5 to 16.0 years. All the children had fever lymph node enlargement, blood system involvement in 74 cases (62.7%), skin injury in 39 cases (33.1%). The main manifestations of laboratory examination were increased erythrocyte sedimentation rate in 90 cases (76.3%), decreased hemoglobin in 58 cases (49.2%), decreased white blood cells in 54 cases (45.8%) and positive antinuclear antibody in 35 cases (29.7%). Ninety-seven cases (82.2%) underwent B-mode ultrasound of lymph nodes, showing nodular lesions with low echo in the neck; 22 cases (18.6%) underwent cervical X-ray and (or) CT; 7 cases (5.9%) underwent cervical magnetic resonance imaging. Lymph node biopsy was performed in all 118 cases, and the pathological results did not support malignant diseases such as lymphoma or Epstein-Barr virus infection, suggesting HNL. Fifty-seven cases (48.3%) recovered without treatment, 61 cases (51.7%) received oral steroid therapy, and 4 cases (3.4%) received indomethacin as anal stopper. The 118 cases were followed up for 4 (2, 6) years, ranging from 1 to 7 years, 87 cases (73.7%) had one onset and did not develop into other rheumatological diseases, and 24 cases (20.3%) had different degrees of recurrence, 7 cases (5.9%) had multiple system injuries, and all of the tested autoantibodies were positive for medium and high titers. All of them developed into other rheumatic immune diseases, among which 5 cases developed into systemic lupus erythematosus and 2 cases developed into Sjogren's syndrome; 7 cases were given oral steroid therapy, including 6 cases plus immunosuppressant and 2 cases receiving methylprednisolone 20 mg/kg shock therapy. Conclusions: The first-onset HNL portion is self-healing, hormone-sensitive and has a good prognosis. For HNL with repeated disease and multiple system injury, antinuclear antibody titer should be monitored during follow-up, and attention should be paid to the possibility of developing into other rheumatological diseases, with poor prognosis.
Female ; Male ; Humans ; Child ; Histiocytic Necrotizing Lymphadenitis/drug therapy* ; Antibodies, Antinuclear ; Epstein-Barr Virus Infections ; Retrospective Studies ; Herpesvirus 4, Human ; Prognosis ; Steroids

Objective: To investigate the economic burden of bacillus Calmette-Guérin (BCG) lymphadenitis in Shandong Province. Methods: From May 2011 to December 2019, 304 patients applying for the province-level compensation of BCG lymphadenitis was selected from Shandong Province in this study. The basic situation, vaccination, outpatient (inpatient) records, cost and relevant information of those patients were collected to calculate the direct economic burden (including direct medical costs and direct non-medical costs), indirect economic burden and total economic burden. Comparison of the difference of economic burden of cases with different characteristics was taken. Results: The M(Q₁,Q₃) of age of BCG lymphadenitis patients was 3 (2, 4) months, among which 239 cases (78.6%) were male, 71 cases (23.4%) had lymphadenopathy, and 227 cases (74.7%) underwent surgery.The number of outpatient only, inpatient only and outpatient then inpatient was 25.7% (78 cases), 7.2% (22 cases) and 67.1% (204 cases), respectively. The M(Q₁,Q₃) of direct, indirect and total economic burden of single case after discount was 9 910 (5 713, 16 074), 2 081 (1 547, 3 122) and 12 262 (7 694, 18 571) yuan, respectively.The direct medical expenses accounted for 89.4% of the direct economic burden, the direct economic burden accounted for 84.9% of the total economic burden, the total economic burden of 80.0% cases accounted for only about 20.0% of the compensation amount, and the total economic burden of only 2.3% cases accounted for more than 60.0% of the compensation amount.The direct, indirect and total economic burden of patients with inpatient only and outpatient then inpatient was higher than that of patients with outpatient only; the direct, indirect and total economic burden of patients with operation was higher than that of patients with non-operation; the direct and total economic burden of patients with unulcerated lymph node was higher than that of patients with ulcerated lymph node(all P values<0.05). Conclusion: The economic burden of BCG lymphadenitis cases in Shandong Province is influenced by the mode of diagnosis and treatment, with direct medical expenses as the predominant component.
BCG Vaccine ; Cost of Illness ; Financial Stress ; Humans ; Infant ; Lymphadenitis/epidemiology* ; Male ; Vaccination

Professor SHAO Jing-ming's clinical experience of fire needling for bone-joint tuberculosis, tuberculous cervical lymphadenitis, ganglion cyst and thyrophyma is summarized. Professor SHAO used fire needling to treat bone-joint tuberculosis. The acupoints included ashi points and nearby acupoints, particularly local opposite acupoints (Neixiyan [EX-LE 4] and Dubi [ST 35], Yinlingquan [SP 9] and Yanglingquan [GB 34], Xuehai [SP 10] and Liangqiu [ST 34]), and for the patients with severe yin-cold syndrome, Yanghe decoction was additionally used. For tuberculous cervical lymphadenitis, fire needling was used at different stages. In the early stage, the nucleus was punctured with fire needling; in the middle stage, the pustule was punctured with fire needling combined with cupping; in the late stage, the fire needling was inserted into the fistula or sinus tract, and the surrounding granulation tissue was treated with horizontal penetrating needling. For ganglion cyst, fire needling combined with centro-square needling was applied. For thyrophyma, the surrounding needling with filiform was used; for simple thyroid mass and thyroid nodule, the surrounding needling with fire needling was used.
Acupuncture Points ; Acupuncture Therapy ; Ganglion Cysts ; Humans ; Lymphadenitis ; Tuberculosis, Osteoarticular

OBJECTIVE: To study the clinical features of children with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, a polygenic and multifactorial autoinflammatory disease with unknown pathogenesis. METHODS: A retrospective analysis was performed on the medical data of 13 children with PFAPA syndrome. RESULTS: All 13 children had disease onset within the age of 3 years, with a mean age of onset of (14±10) months. They all had periodic fever, with 8-18 attacks each year. The mean interictal period of fever was (30±5) days. Pharyngitis, cervical adenitis, and aphthous stomatitis were the three cardinal symptoms, with incidence rates of 100% (13/13), 85% (11/13), and 38% (5/13) respectively. There were increases in white blood cells, C-reactive protein, and erythrocyte sedimentation rate during fever. Of all the 13 children, 6 underwent whole exome sequencing and 7 underwent panel gene detection for autoinflammatory disease, and the results showed single heterozygous mutations in the CONCLUSIONS For children with unexplained periodic fever with early onset accompanied by pharyngitis, cervical adenitis, aphthous stomatitis, elevated inflammatory indices, and good response to glucocorticoids, PFAPA syndrome should be considered. This disorder has good prognosis, and early diagnosis can avoid the long-term repeated use of antibiotics.
Child ; Child, Preschool ; Fever/etiology* ; Humans ; Infant ; Lymphadenitis/diagnosis* ; Pharyngitis/drug therapy* ; Pyrin ; Retrospective Studies ; Stomatitis, Aphthous/genetics*

OBJECTIVE: To evaluate the clinical efficacy of medicinal penetration on acupoint combined with medication for histiocytic necrotizing lymphadenitis (HNL) of heat-toxin syndrome, and to explore the methods of improving the clinical effect. METHODS: A total of 72 cases with HNL with heat-toxin syndrome were randomly divided into an observation group and a control group, 36 cases in each group. The patients in the control group were treated with oral administration of prednisone tablets for 40 days (first 5 days: 10 mg, three times a day; since then, reduced by 5 mg every 7 days). In the observation group, on the basis of the medication in the control group, the patients were treated with acupoint application and ultrasonic drug penetration therapy, once a day for 14 days. The acupoints of Waiguan (TE 5), Fengchi (GB 20) of affected side and points were selected. The changes of target lymph node swelling, visual analogue score (VAS), axillary temperature and total score of symptoms and signs were evaluated before treatment and 7, 14, 28 and 40 d into treatment; the changes of white blood cell (WBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and lactic dehydrogenase (LDH) were evaluated on 14 d and 40 d into treatment; the patients were followed-up for half a year. RESULTS: ① Fourteen days into treatment, the clinical cured rate in the observation group was 38.9% (14/36), which was superior to 16.7% (6/36) in the control group (<0.01); the clinical cured rates were both 100% in the two groups on 40 d into treatment. ② The VAS score, axillary temperature and the total score 7 d into treatment, as well as node swelling, VAS score, axillary temperature and the total score 14, 28 and 40 d into treatment in the two groups were significantly improved (<0.01); the total score and VAS score of 7 d into treatment, target lymph node swelling, VAS score and total score of 14 d into treatment in the observation group were significantly improved than those in the control group (<0.01). ③ Compared before treatment, WBC, CRP, ESR in the two groups were significantly improved 14 d and 40 d into treatment (<0.01), and LDH in the two groups were decreased 40 d into treatment (<0.01), but there was no significant difference between the two groups (>0.05). ④ The recurrence rate in the observation group was 5.6% (2/36), which was similar to 16.7% (6/36) in the control group (>0.05). CONCLUSION The medicinal penetration on acupoint as adjunctive treatment could effectively relieve the discomfort symptoms of HNL patients with syndrome of heat and toxin, improve the clinical cured rate, and provide the research direction for shortening the course of medication.
Acupuncture Points ; Acupuncture Therapy ; Histiocytic Necrotizing Lymphadenitis ; therapy ; Humans ; Medicine, Chinese Traditional ; Prednisone ; therapeutic use ; Treatment Outcome

PURPOSE: This study evaluated the feasibility of acoustic radiation force impulse (ARFI) elastography and characterized the sonographic features of lymph nodes (LNs) with Kikuchi disease in pediatric patients. METHODS: Seventy-six cervical LN biopsies were performed for the diagnosis of cervical lymphadenopathy. ARFI imaging was performed, and the characteristic ultrasound features of the biopsied LNs and the contralateral LNs were analyzed. We also reviewed clinical and conventional ultrasonographic findings. RESULTS: On histology, 56 patients were diagnosed with Kikuchi disease. These LNs were large and elongated, with increased perinodal echogenicity and capsular thickening. In 38 of them, ARFI elastography was performed, and the median shear wave velocity (SWV) of the biopsied LNs with Kikuchi disease (2.19 m/sec; range, 1.45 to 4.57 m/sec) was higher than of the contralateral LNs (1.72 m/sec; range, 0.95 to 2.65 m/sec; P < 0.001). In patients with reactive hyperplasia, the mean SWV of the biopsied LNs (2.00 m/sec; range, 1.49 to 2.26 m/sec) was higher than that of the contralateral LNs (1.55 m/sec; range, 1.21 to 2.32 m/sec; P=0.031). CONCLUSION: The SWV of LNs with Kikuchi disease was significantly higher than that of the contralateral LNs. Morphologically, LNs with Kikuchi disease showed an enlarged, elongated, and oval shape, increased perinodal echogenicity, and capsular thickening. In addition to the conventional ultrasonographic findings, the application of ARFI is feasible even in pediatric patients for the evaluation of cervical lymphadenopathy.
Acoustics* ; Biopsy ; Diagnosis ; Elasticity Imaging Techniques* ; Histiocytic Necrotizing Lymphadenitis* ; Humans ; Hyperplasia ; Lymph Nodes ; Lymphatic Diseases ; Pediatrics ; Ultrasonography

OBJECTIVE: To report a case of a 12-year-old boy with Necrotizing Lymphadenitis (Kikuchi-Fujimoto disease) presenting as lymphadenitis secondary to multiple diagnosis.

METHODS:

Design : Case Report

Setting: Tertiary Private Hospital

Patient: One

RESULTS: A 12-year-old boy consulted for two-month history of cervical lymphadenopathy with the underlying cause remaining unclear despite multiple consults, diagnosis and medical treatment. Lymphoma was considered and excision biopsy with further investigations confirmed a diagnosis of Kikuchi-Fujimoto disease. Supportive management was given with no recurrence of symptoms noted on 18 months of follow up. 

CONCLUSION:  Kikuchi-Fujimoto disease in this case was a diagnosis of exclusion. Even with a proper history and physical examination, experts in otolaryngology can be misled to manage this case as malignant. Awareness of the disease and appropriate examinations including immunohistochemistry are important for a timely diagnosis and proper intervention.

KEYWORDS: lympadenitis; Kikuchi-Fujimoto disease; lymphoma; cervical lymph nodes

PURPOSE: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a leading cause of periodic fever in children. This study describes the clinical characteristics of PFAPA syndrome in patients from a single center.METHODS: Thirteen children diagnosed with PFAPA syndrome at Seoul National University Children's Hospital were included in this study. Retrospective medical chart reviews were performed.RESULTS: Among the 13 patients, 8 (61.5%) were male. The median follow-up duration was 3.3 years (range, 10 months–8.3 years). The median age of periodic fever onset was 3 years (range, 1–6 years). All patients had at least 5 episodes of periodic fever and pharyngitis, managed with oral antibiotics, before diagnosis. The median occurrence of fever was every 3.9 weeks and lasted for 4.2 days. All patients had pharyngitis and 12 (92.3%) had cervical lymphadenitis. Blood tests were performed for 12 patients, and no patients had neutropenia. Both the C-reactive protein and erythrocyte sedimentation rate were elevated at medians of 4.5 mg/dL (range, 0.4–13.2 mg/dL) and 29 mm/hr (range, 16–49 mm/hr), respectively. Throat swab cultures and rapid streptococcal antigen tests were negative. Nine (69.2%) patients received oral prednisolone at a median dose of 0.8 mg/kg, and in 6 (66.7%) patients, fever resolved within a few hours. Three (23.1%) patients received tonsillectomy and adenoidectomy.CONCLUSIONS: PFAPA syndrome should be considered when a child presents with periodic fever along with aphthous stomatitis, pharyngitis, or cervical lymphadenitis. Glucocorticoid administration is effective for fever resolution and can reduce unnecessary use of antibiotics.
Adenoidectomy ; Anti-Bacterial Agents ; Blood Sedimentation ; C-Reactive Protein ; Child ; Diagnosis ; Fever ; Follow-Up Studies ; Hematologic Tests ; Humans ; Lymphadenitis ; Male ; Neutropenia ; Pharyngitis ; Pharynx ; Prednisolone ; Retrospective Studies ; Seoul ; Stomatitis, Aphthous ; Tonsillectomy ; Yemen