An Unusual Cause of Lymphadenopathy: Rosai Dorfman Disease in a 7-Year-Old Female Zambian Child: Case Report and Literature Review.

Rosai Dorfman disease (RDD) is a rare non-Langerhans histiocytic disorder, which belongs to the R group of the 2016 revised histiocytic classification. It's characterized by the accumulation of activated histiocytes in the sinusoids of lymph nodes and/or extranodal tissues. Herein, we report a 7-year-old female who was initially suspected to have a lymphoma but was later identified as having RDD. She presented with a history of fever, night sweats, and weight loss, and on physical examination had bilateral cervical lymphadenopathy. Histologic examination of the biopsied cervical lymph nodes showed distended sinuses with S100 and CD68 immunoreactive histiocytes demonstrating emperipolesis, confirming a diagnosis of RDD. The condition is known to be self-limiting. However, evidence from literature and our case management shows that medical therapy can hasten remission in pediatric cases.

Comparison of adverse events following the second/third dose of BNT162b2 in a medical institute in Japan.

Background: Vaccines against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are crucial for ending the pandemic of coronavirus disease 2019 (COVID-19). Currently, the cumulative effect of booster shots of mRNA vaccines on adverse events is not sufficiently characterized. Methods: A survey-based study on vaccine adverse events was conducted in a Japanese medical institute after the third dose of Pfizer BNT162b2. Adverse events were grouped using network analysis, and a heteroscedastic probit model was built to analyse adverse events. Results: There were two main clusters of adverse events, systemic and local injection site-associated events. Subject background and the experience of previous vaccine-related adverse events were variably associated with the occurrence and intensity of adverse events following the third dose. Among adverse events, only lymphadenopathy increased prominently following the third dose, while the largest increase in other systemic adverse events occurred generally following the second dose. Conclusions: The effect of repeated booster vaccines on the frequency and intensity of adverse events differs depending on the kind of adverse event.

Generalized lymphadenopathy in the presence of acute Epstein-Barr virus infection as the initial manifestation of systemic lupus erythematous: A case report.

Key Clinical Message: Clinicians should carefully consider generalized lymphadenopathy, particularly post viral infections, as one of the possible systemic lupus erythematous (SLE) first signs regarding unusual joint involvements such as sacroiliitis. Late diagnosis of this autoimmune inflammatory disease, could lead to irreversible morbidity and higher mortality. Abstract: Lymphadenopathy could represent various etiologies, including infections, malignancies, and rheumatologic diseases. SLE is known as the great mimicker which could be presented with different first manifestations. We report a 42-year-old woman in the acute phase of Epstein-Barr infection, admitted with polyarticular peripheral arthritis, sacroiliitis, and generalized lymphadenopathy. She had no similar history or taken unpasteurized dairy. Nodes were soft, mobile, and tender without skin change on top. During the process, she was diagnosed with SLE and discharged with prednisolone 30 mg/day and hydroxychloroquine 400 mg/day. After 2 weeks of follow-up, all lymphadenopathy and symptoms were diminished. This case underscores the thousand faces innate of SLE. Clinical awareness would lead to an accurate diagnosis and early intervention.

Advanced human immunodeficiency virus (HIV) does not affect ability to utilize lymphadenopathy in assessment of drug reaction with eosinophilia and systemic symptoms syndrome in HIV and tuberculosis: Prospective comparative study.

Background: RegiSCAR validation criteria for drug reaction with eosinophilia and systemic symptoms (DRESS) includes lymphadenopathy, a frequent feature of both tuberculosis (TB) and human immunodeficiency virus (HIV). TB is the most common HIV-associated coinfection. Advanced HIV is associated with lymph node (LN) fibrosis. It is not clear if this negatively affects case validation in HIV-associated DRESS. To answer this question, we designed a prospective descriptive study to assess lymphadenopathy in various combinations of comorbid HIV, TB, and DRESS. Objectives: We sought to describe the prevalence of DRESS-associated lymphadenopathy and characterize LN quality, size, and distribution in a high HIV-TB burden setting over time. Methods: We prospectively and systematically examined LN in 25 consecutive acute DRESS cases hospitalized at a South African tertiary-care center and 10 hospitalized non-DRESS HIV-TB coinfected controls. Results: Fourteen (56%) of 25 patients were HIV infected, with a median (interquartile range) CD4 count of 254 (66-478) cells/mm³, and 7 of 14 were coinfected with TB. Using RegiSCAR criteria, 12 (46%) of 25 were definite DRESS cases, 8 (31%) of 25 probable, and 5 (23%) of 25 possible. Possible cases were excluded in the analysis. Fifteen (75%) of 20 subjects had LN in ≥2 anatomic sites, including all 7 patients with HIV-TB coinfection. In contrast, 1 (20%) of 5 hospitalized non-DRESS HIV-TB coinfected controls had LN. Cervical LN, in 15 (88%) of 17, was most common, followed by axillary (76%) and inguinal (59%). Cervical LN ranged between 1 and 2 cm in size. Among the 8 (32%) of 25 subjects with follow-up data, LN had regressed in all within 6 weeks of stopping the offending drug and initiating TB treatment. There was no correlation with CD4 cell count and LN. Conclusion: Lymphadenopathy is a common feature of acute DRESS, even among HIV-TB-coinfected patients with advanced immunosuppression.

A Case Report on a Rare Type of Lymphoma: Angioimmunoblastic T-cell Lymphoma.

Angioimmunoblastic T-cell lymphoma (AITL) is a rare type of non-Hodgkin lymphoma (NHL). We present a case of a 60-year-old female who attended the emergency department (ED) with fatigue, recurrent fever, weight loss, and adenopathy for six months. Laboratory findings showed anemia, lymphocytosis, eosinophilia, thrombocytosis, cholestasis, hypoproteinemia, and hypoalbuminemia. Abdominopelvic computed tomography (CT) revealed multiple adenopathies. A lymph node biopsy yielded inconclusive results in the outpatient clinic. Later, during admission, the patient underwent a positron emission tomography-computed tomography (PET-CT), revealing a cervical adenopathy cluster that was excised en bloc. Histology confirmed the diagnosis of AITL. The medical team initiated chemotherapy but opted for exclusive symptomatic treatment due to disease progression. The patient died six months after diagnosis. The fluctuating and nonspecific presentation of AITL can hinder and delay definitive diagnosis, therefore impacting treatment and prognosis.

Recurrence of Cervical Lymph Node Metastasis From an Unknown Primary Site: A Report of a Case Treated Using a Multidisciplinary Approach.

Metastatic cervical carcinoma from an unknown primary source poses a diagnostic and therapeutic challenge, as it involves the spread of cancer to the neck lymph nodes without a discernible primary tumor despite thorough investigation. While the diagnosis and treatment of this uncommon condition lack definitive evidence, a review of existing literature offers some clinical guidance. A comprehensive diagnostic evaluation, which includes multiple imaging and endoscopic studies, is essential. Surgery is preferred whenever feasible due to its ability to offer more precise staging. This treatment entails an excisional biopsy, neck dissection, and tonsillectomy, but advanced cases necessitate a combination of treatments. This case report underscores this complexity, where, despite radical neck dissection on the affected side, recurrence manifested after two months with no discernible primary site. We emphasize the urgency for continued research and innovative approaches to enhance the diagnosis and management of metastatic cervical carcinoma from an unknown primary source.

Disseminated AIDS-associated Kaposi sarcoma without cutaneous involvement: a case report and review of literature.

Disseminated AIDS-associated Kaposi sarcoma (KS) without cutaneous lesions is rare and can present in varying ways. Diagnosis is even more challenging now when incidence of KS is on the decline. A high index of suspicion is required for early diagnosis and treatment. Therefore, the medical literature should be made aware of any manifestations of KS that can occur without the typical cutaneous lesions. A 23-year-old presented with worsening cervical lymphadenopathy, recurrent cough and bilateral leg swelling of a month duration. Examination revealed features of pericardial effusion, pulmonary fibrosis, necrotizing cervical lymphadenopathy and the presence of pityriasis rotunda at the periumbilical region. Patient was diagnosed human immunodeficiency virus (HIV) positive 6 months before she presented and was placed on antiretroviral therapy. Histology confirmed AIDS-associated KS. However, patient died before commencement of chemotherapy. The clinical course of disseminated AIDS-associated KS without cutaneous lesions can be atypical and aggressive. It is important to include KS in the differential diagnosis of cases with atypical or persistence/recurrence of clinical symptoms in spite of treatment especially in HIV patients.

Comparison of pathophysiological differences in TAFRO syndrome with or without lymphadenopathy.

In diagnosing TAFRO syndrome, lymph node histology often may not be evaluated due to inapparent lymphadenopathy. In this study, we analyzed the differences in the pathophysiology of TAFRO syndrome with or without lymphadenopathy. We used an anonymous questionnaire to survey 70 hematologists at 50 hospitals in the Kanto Koshinetsu area of Japan from February to April 2020. We received 31 responses and collected 26 cases with TAFRO syndrome. Compared to cases with or without lymph node biopsy, clinical features and laboratory test findings in both groups were not significantly different, except for stronger renal insufficiency found in those without biopsy. It was also revealed that clinical features and laboratory test findings had no significant differences between the cases with and without lymphadenopathy. However, renal insufficiency was more pronounced in those without lymphadenopathy. There were no significant differences in pathophysiology between cases with or without lymphadenopathy in the group that did not undergo lymph node biopsy. In the treatment strategies, no significant differences were found dependent on lymphadenopathy. This study shows that lymphadenopathy in TAFRO syndrome may be secondary to inflammation and unrelated to the underlying disease.

A Comparison of Transoral Versus Transcervical Surgical Approaches to Retropharyngeal Lymphadenectomy: A Scoping Review.

IMPORTANCE: A gap in knowledge exists concerning the functional outcomes and complications when comparing various surgical approaches for retropharyngeal lymph node (RPLN) metastases. OBJECTIVE: To explore perioperative outcomes, functional outcomes, and complications associated in the treatment of RPLN metastases. DESIGN: The Preferred Reporting Items for Systematic Reviews and Meta-Analysis extension for Scoping Reviews (PRISMA-ScR) protocol was used to conduct a scoping review of the PubMed and Scopus databases. REVIEW METHODS: We systematically searched 2 databases from inception to January 2023 for articles examining the treatment approaches and postoperative outcomes in the retropharyngeal space. We included English records about surgical approaches, complications, functional outcomes for patients >18 years old with retropharyngeal lymphadenopathy. RESULTS: One-hundred ninety-nine articles were identified, of which 17 were included in the analysis. Three studies assessed RPLN dissection in the postradiation setting. We identified limited knowledge about functional outcomes and complications following surgery for retropharyngeal lymphadenopathy. Overall, acute postoperative dysphagia was documented in 35/170 patients (20.5%). However, the assessment of dysphagia was limited, and not described in the majority of studies. The overall rate of postoperative neuropathy and hematoma were 4.1% and 4.7%, respectively. No postoperative hematomas were documented in the transcervical approach. CONCLUSION: Our findings underscore the need for further research on postoperative outcomes following RPLN dissection. We recommend further studies focusing on objective swallow assessments and long-term outcomes of either surgical approaches.

Diagnostic Accuracy of Fine-Needle Aspiration Cytology in Cervical Lymphadenopathies at a Tertiary Care Center in the Kingdom of Bahrain.

Introduction  Fine-needle aspiration cytology (FNAC) has become widely used as a first-line diagnostic tool in the evaluation of cervical lymphadenopathies (LADs). However, there are conflicting reports regarding its accuracy in differentiating between malignant and benign pathologies. In this study, we aim to determine the reliability of FNAC in distinguishing between benign and malignant pathologies causing cervical LAD. Methods This is a cross-sectional study reviewing the electronic medical records of all patients who underwent both FNAC and excisional biopsy of cervical LADs between January 2016 and December 2023 at a tertiary care center in the Kingdom of Bahrain. A comparison was conducted between the cytopathological results obtained by FNAC and the histopathological results obtained by excisional biopsy to determine the diagnostic accuracy of FNAC. Results In the study period, 83 patient records were reviewed and included in the data analysis. Fine-needle aspiration cytology yielded a sensitivity of 89.3%, a specificity of 55.6%, a positive predictive value (PPV) of 72.4%, a negative predictive value (NPV) of 80.0%, and an overall accuracy of 74.7% in diagnosing cervical LADs. Conclusion Despite FNAC being accessible, convenient, and cost-effective, it has certain limitations that can restrict its accuracy in diagnosing lymphomas. We recommend further studies to research these limitations and the possible tools, such as ancillary testing, that may be useful in overcoming them.

Kikuchi Disease "A Lupus Mimicker": A Case Report.

Kikuchi disease, also called, Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis, is a rare self-limiting illness with an unknown etiology and pathogenesis. It is predominantly seen among young females. The cardinal clinical features include fever and cervical lymphadenopathy. Skin eruptions have also been reported. In Oman, two cases have been reported to date, in 2005 and 2020, with only one case exhibiting skin manifestations. There is a paucity of disease in our country and worldwide. In this case report, we discuss the diagnosis of Kikuchi disease in a previously healthy 17-year-old Omani female who presented with fever, cervical lymphadenopathy, and malar rash. The clinical picture in this case resembled that of systemic lupus erythematosus. Due to the rarity of Kikuchi disease, particularly in our region, it is crucial to consider it as a differential diagnosis when a patient exhibits the aforementioned symptoms to prevent misdiagnosis and inappropriate treatment, as it can easily be misdiagnosed as systemic lupus erythematosus.

Herpes Simplex Virus Necrotic Lymphadenitis Masquerading as Lymphoma.

In immunocompromised patients, the rapid development of lymphadenopathy could pose a few diagnostic challenges. This is important, especially with chronic lymphocytic leukemia and small lymphocytic lymphoma (CLL/SLL) which can manifest with the development of infections or may even progress with transformation into a more aggressive form of the disease. We report a case of a patient with CLL/SLL who presented with fever and worsening dyspnea as well as inguinal lymphadenopathy upon evaluation. The excisional biopsy of affected lymph nodes revealed herpes simplex virus lymphadenitis confirmed by immunohistochemical staining. Flow cytometry showed no progression to diffuse large B-cell lymphoma. This case highlights the importance of considering a broad spectrum of differential diagnoses when assessing lymphadenopathy in immunocompromised patients receiving active immunosuppressive therapy.

Severe thrombocytopenia caused by cytomegalovirus infection in an immunocompetent adult: A case report.

A 53-year-old otherwise healthy man was referred to our hospital with a fever of unknown origin, headache, and arthralgia. Four days earlier, he had a fever with chills. Treatment with antibiotics and acetaminophen proved ineffective, with the patient subsequently developing headache and joint pain. Blood analysis revealed elevated inflammatory markers, liver impairment, and severe thrombocytopenia (platelet count, 19,000/µL). Subsequent tests revealed elevated levels of anti-cytomegalovirus IgM and IgG. Based on these findings, the patient was diagnosed with severe thrombocytopenia associated with cytomegalovirus infection. Platelet counts increased spontaneously without antiviral therapy. Forty-five days after the initial visit, the symptoms improved, and blood tests revealed resolution of the inflammatory findings, with the platelet count recovering to 155,000/µL. Although the disease may resolve spontaneously, cytomegalovirus infection should be considered as a differential diagnosis in case of severe thrombocytopenia in immunocompetent adults.

Clinical prediction model for pediatric lymphadenopathy: enhancing diagnostic precision and treatment decision making.

INTRODUCTION: Lymph node enlargement is common in children, with 90% of physiologically palpable lymph nodes. This study aimed to develop a predictive model based on clinical characteristics to enhance the diagnosis of pediatric lymphadenopathy and provide insights into biopsy outcomes. MATERIALS AND METHODS: A clinical prediction rule was developed using a retrospective, cross-sectional design for patients under 15 years who underwent lymph node biopsy from 2012 to 2022. Multivariable risk regression was used to analyze benign and malignant lesions, presenting results through risk difference and AUROC for each group. Predicted probabilities were applied in a logistic regression equation to classify patients' lymphadenopathy as reactive hyperplasia, benign, or malignant. RESULTS: Of 188 children, 70 (37.2%) had benign lymphadenopathy beyond reactive hyperplasia, and 27 (14.4%) had malignant lymphadenopathy. The predictive model included 12 characteristics such as size, location, duration, associated symptoms, and lymph node examination. Predictive accuracy was 92.2% for benign cases (AUROC = 0.92; 95% CI 0.87-0.96) and 98.6% for malignancy (AUROC = 0.98; 95% CI 0.94-0.99). Overall accuracy for predicting both benign and malignant tumors was 68.3%. CONCLUSION: The model demonstrated reasonably accurate predictions for the clinical characteristics of pediatric lymphadenopathy. It tended to overestimate malignancy but did not miss diagnoses, aiding in reducing unnecessary lymph node biopsies in benign cases.

Role of 18F-FDG PET/CT in the management of head and neck cancer patients with persistent cervical lymphadenopathy following chemoradiation.

BACKGROUND: We aim to describe the management and outcomes of patients with persistent lymphadenopathy (LAD) after primary chemoradiation for head and neck squamous cell carcinoma (HNSCC) based on post-treatment PET/CT results. METHODS: Retrospective chart review was conducted of all patients who underwent primary concurrent chemoradiation for HNSCC at a tertiary care center from 2010 to 2022 and had persistent post-treatment LAD. RESULTS: Nearly 62% of patients were managed conservatively, and 27.0% underwent neck dissection. PET-positive patients were more likely to undergo neck dissection than PET-negative patients (p = 0.042). Positive predictive value (PPV) and negative predictive values (NPV) of PET/CT in detecting residual disease in the neck were 48.0% and 73.7%, respectively. CONCLUSIONS: PPV and NPV of PET/CT for detecting residual neck disease in patients with post-treatment LAD was lower than those of HNSCC patients with and without persistent LAD reported in other studies.

Rosai-Dorfman Disease as Constrictive Pericarditis: An Unusual Childhood Presentation.

Rosai-Dorfman disease (RDD) is a rare, multisystemic, histiocytic disorder that usually presents with painless cervical lymphadenopathy. We describe a case of constrictive effusive pericarditis with congestive cardiac failure in a six-year-old child as an initial presentation of RDD. The child underwent pericardiectomy and was treated with steroids, following which the symptoms resolved entirely. While tuberculosis accounts for the majority of cases presenting with constrictive pericarditis in developing countries, the presentation of RDD can be easily missed if not kept in mind as a possibility. This case report documents the unique presentation of a rare disease.

Silicone Granulomas in the Orbit following Breast Implant Rupture: Case Report.

Introduction: A known but uncommon complication following breast augmentation with silicone implants is the rupture of these implants and subsequent silicone migration through the lymphatic system. Exceptionally, there are sporadic instances of silicone granulomas forming in distant, non-lymphatic sites, posing diagnostic and management challenges in clinical practice. Case Presentation: A 56-year-old woman presented with slowly progressive diplopia and photosensitivity during the past 12 months. Ophthalmic examination revealed restriction of movement in all gazes in the right eye. Investigation with magnetic resonance imaging and positron emission tomography-computed tomography showed enlarged superior lateral and inferior rectus muscles in the right orbit, and a diffusely enlarged lacrimal gland in the left orbit, as well as a ruptured silicone breast implant on the right side. In addition, multiple enlarged lymph nodes were found throughout the body, as well as a mass in the internal oblique muscle of the abdominal wall. Fine-needle aspiration biopsy of the axillary lymph node and surgical biopsy of extraocular muscles confirmed a diagnosis of silicone granulomas. The patient received anti-inflammatory treatment with intravenous steroids but with no effect on symptoms. Conclusions: This case illustrates a rare instance of silicone dissemination from a ruptured breast implant leading to granuloma formation in multiple organs, including the orbit. Notably, the spread of silicone appeared to occur through both lymphatic and hematogenous routes. This finding underscores the importance of considering silicone granulomas in the differential diagnosis of orbital lesions for patients with a history of silicone implants.

Multiple foci of Rosai-Dorfman disease in colon: a case report.

BACKGROUND: Rosai-Dorfman disease (RDD) is an uncommon proliferative histiocytic disorder involving lymph nodes and various organs. Forty-three percent of RDD cases originate from extranodal sites; however, RDD rarely arises from the colon. CASE PRESENTATION: A 75-year-old man was admitted to our hospital because of intra-abdominal masses that were incidentally detected during surveillance by computed tomography (CT) after treatment for lung cancer. Enhanced CT showed two mass lesions located in the cecum to the appendix (diameter, 40 mm) and around the sigmoid colon (diameter, 24 mm). Positron emission tomography (PET)-CT revealed an apparent uptake of fluorodeoxyglucose. Intraluminal endoscopy did not reveal definite mucosal abnormalities. These findings suggest the presence of malignant neoplasms including gastrointestinal stromal tumors, lung cancer metastasis, and malignant lymphoma. Exploratory laparoscopy and/or tumor excision were planned to obtain a definitive diagnosis. Based on laparoscopic findings, ileocecal resection and sigmoidectomy were simultaneously performed to excise the tumors. Postoperative histopathological examination revealed multiple RDD originating from the mesocolon side of the cecum and the sigmoid colon. The patient did not receive any adjuvant therapy. No recurrence was observed one year after surgery. CONCLUSION: RDD originating from the colon is extremely rare. Tumor extirpation or organ resection is sometimes required to obtain a definitive diagnosis of RDD, and minimally invasive surgery is helpful.

Mpox-Induced Proctitis.

Introduction: Mpox, formerly known as monkeypox, is a zoonotic virus in the genus Orthopoxvirus, which has a variable incubation period and an extensive array of symptoms. While those infected with Mpox have displayed generalized viral prodromal symptoms, atypical symptoms such as proctitis have also been seen. Proctitis associated with Mpox is a relatively infrequent initial presenting symptom with a reported incidence of 14-32.9% that has seen an uptick in prevalence since the 2022 global endemic. Case Presentation: We present a confirmed case of Mpox in a 27-year-old male who presented with 3 days of intermittent anorectal bleeding and various forms of cutaneous lesions at different stages of healing. He had engaged in unprotected sexual intercourse 8 days prior to the onset of his symptoms in New York, which at the time was the epicenter of the endemic. Computed tomography imaging showed thickening of the rectum with associated lymphadenopathy, consistent with findings of acute proctitis. Conclusion: The intent of this case report is to acknowledge the prevalence of the Mpox virus. Since the endemic, increased cases of Mpox have led to more complications that have been identified and studied by public health experts. The complication of proctitis due to Mpox in a certain subset of patients is important to fully understand that while this virus presents with a generalized prodrome like other viruses, these unique gastrointestinal presentations and findings may be the first step in identifying this infection and ensuring rapid treatment if future endemics arrive.