Kikuchi-Fujimoto Disease Research Articles

THE DIAGNOSTIC DILEMMA: KIKUCHI FUJIMOTO DISEASE; A CASE REPORT

TYPE: Abstract TOPIC: Respiratory Care PURPOSE: Kikuchi Fujimoto Disease (KFD) is a rare condition. Handful of cases have been reported in literature. Studying and reporting KFD cases could enable clinicians in timely management of patients. METHODS: An 18 year old male referred to the Respiratory outpatient clinic from Emergency department following a mechanical fall while skating with right cervical lymphadenopathy and generalised pain. Blood tests: Unremarkable. Cervical X ray: Unremarkable. MRI scan: Conglomerate enlarged right level V lymph nodes (inflammatory/ granulomatous in aetiology). CT scan Neck and Chest:Right posterior cervical lymphadenopathy. US guided biopsy: Right neck lymph node (1.5x4cm). Negative for ZN stain. Serology sampling: CMV and HIV negative. Smear test: Negative for AFB showing necrotizing granulomatous lymphadenitis. Primary B cell follicles were CD20, CD79a, CD3 positive. T cells, macrophages were CD68 and myeloperoxidase positive. EBV (EBER) was negative. Anti-EBV capsid IgG and IgM were however detected. HPV B19 IgM was also detected. QuantiFERON TB gold assay test: to exclude latent TB infection was negative. RESULTS: Based on the extensive investigation panel, it was thus formally concluded that the clinical features and results were consistent with the rare diagnosis of Kikuchi's lymphadenitis. CONCLUSIONS: KFD is a very rare disease of benign nature. Cause being unknown, treatment is mainly symptomatic with the use of analgesics such as NSAIDS. Occasionally steroids are used. CLINICAL IMPLICATIONS: Patients presenting with KFD may go on to developing SLE later in life. Due to limited data, it becomes difficult to establish a clear link between the two conditions. Further studies on similarly presenting patients are hence encouraged. DISCLOSURE: Nothing to declare. KEYWORD: Kikuchi Fujimoto Disease (KFD)

Kikuchi-Fujimoto Disease: An Experience from a Tertiary Care Center in South India

Kikuchi-Fujimoto Disease: An Experience from a Tertiary Care Center in South India

Kikuchi-Fujimoto disease in a child who had a high suspicion of COVID-19 infection.

Kikuchi-Fujimoto disease in a child who had a high suspicion of COVID-19 infection.

Kikuchi Fujimoto disease: a rare case report

Kikuchi disease, also known as Kikuchi histiocytic necrotizing lymphadenitis, was initially described in young Japanese women. It is a rare benign self-limiting disease of unknown cause usually characterized by fever and cervical lunphadenitis. It is diagnosed by doing excisional biopsy of affected lymph node. A case of young 20 years old female presented with complaint of fever and weight loss for 20 days along with cervical and axillary lymphadenopathy. Complete physical examination, radiological investigations and biochemical tests were done to rule out systemic lupus erythematous, non-Hodgkin lymphoma and tuberculosis. Only symptomatic treatment was done along with corticosteroids. Its diagnosis is important as it can be easily mistaken for other form of lymphadenitis. Clinician and pathologists’ awareness of this disorder is very necessary.

A Malignant Mimicker: Features of Kikuchi-Fujimoto Disease in the Pediatric Population.

Kikuchi-Fujimoto disease (KFD) is a rare, benign, and self-limited disease that presents with cervical lymphadenopathy and systemic symptoms. Histologic evaluation is often necessary to differentiate KFD from other entities. Electronic medical records and diagnostic material were reviewed for 14 children diagnosed with KFD and 6 children diagnosed with infectious mononucleosis (IM) from 2013-2021. Four cases of KFD were further characterized using targeted DNA-based next-generation sequencing. Systemic symptoms were present in 86% (n = 12/14) of KFD patients, the most common being fever. Laboratory values worrisome for malignancy included cytopenia(s) (n = 9/12), elevated ESR and/or CRP (n = 9/12), elevated ferritin (n = 7/7), and elevated LDH (n = 7/10). Histologically, lymph nodes showed characteristic necrotic foci without neutrophils surrounded by MPO+ "crescentic" histiocytes. Immunoblasts and CD123+ plasmacytoid dendritic cells (pDCs) were also increased surrounding the necrosis. IM lymph nodes showed similar features when necrosis was present but increases in pDCs were patchy and rare neutrophils were seen in the necrotic foci. Molecular analysis of 4 KFD cases did not identify pathogenic variants. While the signs/symptoms of KFD are worrisome, there are pathologic features that help differentiate it from potential mimics. We did not identify characteristic molecular features to aid in the work-up of these cases.

Chronic Fever with Recurent Cervical Cystic Lymphadenitis as Long COVID-19 Alertness to Kikuchi-Fujimoto Disease and Autoimmune Disease in the Future

Long COVID-19 are becoming more common and may continue to rise in the future. It is important to be aware of the emergence of hyperimmune responses and autoimmune diseases following COVID-19. Infection, including viral infections, has been linked to the onset of autoimmune diseases. A 31-year-old woman who works as front officer of a COVID-19 clinic experienced signs and symptoms of long COVID-19, manifested as sterile chronic fever and recurrent cervical cystic lymphadenitis and several other symptoms that suggest a hyperimmune response. Lymph node histology revealed cystic appearance and histiocyte dominance with starry sky formation, that made the suspicion of Kikuchi-Fujimoto disease. Treatment with steroids temporarily improves symptoms, and hydroxychloroquine stops all symptoms and signs. Follow-up of this case is necessary due to its association with autoimmune disease in the future.

Kikuchi-Fujimoto disease in a tertiary care teaching hospital in Coastal South India: A 8-year retrospective study.

A retrospective hospital-based descriptive cross-sectional study was conducted in tertiary care hospitals from 2011 to 2019. All patients with confirmed KFD diagnosis were included and after ethics committee approval the clinical details and histopathological data was retrieved from the medical records department and analyzed. A total of 67 cases were included. The mean age was 26.9±11.3 years with a female: male ratio of 1.9:1. There were 50 patients with tender cervical lymphadenopathy which was the most common clinical presentation. The mean length and width of palpable lymph nodes were 2.3±1.0 cm and 2.2±0.7 cm respectively. Histology revealed proliferative stage in majority of patients ( n=40, 59.7%). Lymphadenopathy resolved in 83.6% within 2 months. There were 42 patients who had complete recovery with symptomatic treatment within a period of 9 months. KFD is prevalent in young, female patients of Asian descent and often presents as tender cervical lymphadenopathy. Early diagnosis with excisional lymph node biopsy is crucial to avoid unnecessary investigations and treatment. Treatment is symptomatic unless complicated, where steroid therapy is considered. KFD has an excellent prognosis with almost no risk of fatality.

Kikuchi-Fujimoto disease: potential immune-mediated pathogenesis, a rare case and literature review

Kikuchi-Fujimoto disease: potential immune-mediated pathogenesis, a rare case and literature review

Blood cell and marrow changes in patients with Kikuchi disease.

Not available.

Kikuchi-Fujimoto disease is mediated by an aberrant type I interferon response

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.

Uncommon presentation of Kikuchi disease.

Kikuchi disease is common among Asians and women aged about 30 years. General symptoms include cervical lymphadenopathy and fever; 64% of the cases are self‐limiting. However, this case is axillary lymphadenopathy, which is the main symptom in males. Thus, Kikuchi disease may be considered in the differential diagnosis of axillary lymphadenopathy.

Kikuchi-Fujimoto Disease (KFD) in a Young Boy with Cervical Lymphadenopathy

Kikuchi-Fujimoto Disease (KFD) in a Young Boy with Cervical Lymphadenopathy

The fine-needle aspiration cytology and clinical findings of Kikuchi–Fujimoto disease in pediatric patients: a retrospective clinical study

Background Histological evaluation of lymph node is crucial for the definitive diagnosis of Kikuchi–Fujimoto disease (KFD). However, lymph node biopsy under local anesthesia is often difficult in pediatric patients. Objectives We evaluated cytological findings for pediatric patients with prolonged cervical lymphadenitis clinically suggestive of KFD and investigated the clinical characteristics of patients diagnosed with KFD by fine-needle aspiration cytology (FNAC). Methods This retrospective clinical study included 58 Japanese pediatric patients with cervical lymphadenitis who underwent FNAC. Results Cytological diagnosis was KFD for 22 and suspicion of KFD for 11 patients. The remaining 25 patients were diagnosed with non-specific lymphadenitis (NSL). Tenderness was independently associated with a higher frequency of both KFD in narrow and broad senses, compared with NSL (p = .009; p = .038). The percentage of patients who underwent FNAC within 28 days from symptom onset tended to be higher among patients with KFD in a narrow sense than those with NSL (p = .052). Conclusion This study indicated that the period from symptom onset to FNAC (<28 days) and the symptom of tenderness were associated with the cytological diagnosis of KFD.

Kikuchi Disease Masquerading as a T-Cell Lymphoma

Abstract Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is most prevalent in Asia and has been increasingly recognized in other parts of the world. It usually occurs more frequently in females and presents as fever and localized cervical lymphadenopathy. It resolves spontaneously, usually over a period of several weeks up to 6 months. Its initial clinical features are commonly similar to that of a lymphoma, and it can be misdiagnosed both by clinicians and pathologists. Kikuchi disease has been associated with systemic lupus erythematosus. We report a case of Kikuchi disease that occurred in a 25-year-old female patient that masqueraded as a T-cell lymphoma.

Clinical Analysis of 44 Children with Subacute Necrotizing Lymphadenitis.

ObjectiveTo explore the causes, clinical features, and laboratory examination characteristics of subacute necrotizing lymphadenitis to improve the understanding of this disease, reduce misdiagnosis, and make appropriate diagnoses and treatments.MethodsThe clinical data of 44 children with subacute necrotizing lymphadenitis admitted to our hospital from January 2013 to August 2021 were collected. The clinical and laboratory examinations of the disease were systematically analyzed.ResultsAll 44 cases aged 6–13 years and averaged 10.3 ± 2.1 years, including 32 boys and 12 girls, were diagnosed with histiocytic necrotizing lymphadenitis (HNL) by lymph node biopsy. Forty-two cases had palpable superficial lymph nodes, and two showed abdominal lymph node enlargement in the imaging examination. The symptoms might be accompanied by mild hepatomegaly or splenomegaly, emaciation, night sweats, rashes, and other manifestations. Leukopenia and neutropenia occurred. The pathological features were extensive coagulative necrosis of lymph nodes with reactive hyperplasia of histiocytes as well as positive IHCl CD68. Four cases had a relapse, and the prognosis of the other cases was good.ConclusionThe case with fever and lymph node enlargement of unknown origin and no response to antibiotic treatment should undergo a pathological examination as early as possible for diagnosis and treatment. The disease has a good prognosis in most cases but may recur in a few cases.

Kikuchi-Fujimoto disease and systemic lupus erythematosus – an enigmatic association for clinicians and pathologists: Case report and short literature review

Abstract Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a benign condition of the lymphatic tissue whose cause has not been fully elucidated. Although it has been thought that various viral infections are involved in the onset of the disease, its immunological characteristics expressed by the proliferation of cytotoxic CD8-positive T lymphocytes and histiocytic phagocytosis place it at the border of autoimmunity. Sharing immunological mechanisms, clinical manifestations, and epidemiological data (both are described in young women) with systemic lupus erythematosus (SLE), the association of the two pathologies represents a challenge for both clinicians and pathologists. We present the case of a young woman with SLE with immunological, haematological and articular involvement, who associates a few months after diagnosis the significant swelling of the submandibular glands and latero-cervical lymphadenopathy. Histopathological and immunohistochemical examinations subsequently certify the diagnosis of histiocytic necrotizing lymphadenitis associated with the autoimmune disease.

Kikuchi’s disease (KD); mimicking malignant lymphoma: A rare cause of cervical lymphadenopathy.

Objective: The aim of this study is to present six cases of cervical lymphadenopathy, diagnosed as Kikuchi’s disease and raise the index of suspicion for this disease. Study Design: Case Series. Setting: Histopathology and Otolaryngology/Head &amp; Neck Surgery Department at Rehman Medical Institute (RMI) Peshawar. Period: July 2016 to March 2021. Material &amp; Methods: Records of patients for cases diagnosed as “histiocytic necrotising lymphadenitis” were retrieved, their corresponding patient files accessed from the archive, and case notes analyzed. Results: In this series, a total of six cases of Kikuchi’s disease are presented. A female predominance is evident from the present data (2:1 female to male ratio). All patients presented with palpable lymph nodes in the neck, predominantly on the left (66.7%). Majority (66.7%) presented with neuralgic pain (on/off). Only one patient did not show weight loss and night sweating, the remaining had all the symptoms. All of the patients recovered spontaneously within 6 months (range 2 to 6 months) with symptomatic treatment for neuralgic pain. Conclusion: Kikuchi’s disease should be kept in the differential diagnosis in young females with persistent neck masses, palpable lymph nodes and suspicious fine needle aspiration cytology (FNAC).

Differential Diagnosis of Histiocytic Necrotizing Lymphadenitis and Malignant Lymphoma with Simple Clinical Findings.

It is desirable that noninvasive differential diagnosis takes place without lymph node biopsy for histiocytic necrotizing lymphadenitis (HNL) or malignant lymphoma (ML). In this study, we propose a novel scoring model for the differential diagnosis of these diseases using clinical information and clinical findings. We retrospectively analyzed the data from 15 HNL and 13 ML pediatric patients. First, a univariate analysis identified 14 clinical factors with significant differences. Second, a subsequent analysis using receiver operating characteristic (ROC) curve analysis identified three factors among them with area under the ROC curve values of >0.95: body temperature (°C), maximum lymph node size (cm), and serum β2-microglobulin level (mg/L). Finally, the cut-off values of each of these three factors were determined and examined for the 28 cases. All 15 HNL cases were within 2–3 of the cut-off values among the three factors, no ML case was within two or more cut-off values. Thus, the diagnostic sensitivity and specificity of this novel scoring system were both 100%, indicating that clinical scoring with body temperature, maximum lymph node size, and β2-microglobulin are useful for distinguishing between HNL and ML.

Kikuchi-Fujimoto disease (KFD): A Case Report

Abstract Kikuchi-Fujimoto disease (KFD) is a rare entity characterized by subacute necrotizing lymphadenopathy. Despite many studies in the literature, the cause of KFD remains uncertain. Young adults of Asian ancestry are most affected, but it has been reported worldwide. Clinically, KFD is characterized by lymphadenitis of one or more lymph nodes, predominantly in the posterior cervical region, fever, and leukopenia in up to 50% of cases. In this case report, we are presenting a case of Kikuchi-Fujimoto disease in a young Filipino lady. Keywords: Kikuchi-Fujimoto disease, KFD, Kikuchi disease, Acute necrotizing lymphadenopathy, Lymphadenitis.

Histiocytic Necrotizing Lymphadenitis: A Clinical Study of 6 Children and Review

Objective: As a rare cause of fever and lymphadenopathy (especially in the neck) in children, the purpose of this study is to explore the clinical characteristics of histiocytic necrotizing lymphadenitis (HNL) in children and improve the diagnosis and treatment of this disease. Methods: We retrospectively collected the data of 6 pediatric patients with HNL diagnosed by lymph node biopsy from 2012 to 2021 in department of pediatric, the affiliated Changsha Central Hospital, Hengyang Medical School, University of South China, including the clinical characteristics, laboratory examinations, imaging findings and histopathological examinations, treatment and follow-up of the patients and reviewed relevant literature. Results: All the patients were Han girls, with no kinship with each other, and four cases were from rural areas. The age of onset was from 7 to 14 years old, with durations ranged from 1 week to 3 months. All children had cervical lymphadenopathy with tenderness, five cases of fever, two cases of cough, one case of weight loss, four cases of leukopenia, one case of liver impairment, one case of combined bacterial infection of leukocytosis. All children underwent lymph node biopsy and were diagnosed with HNL, four children were treated with anti-infection before diagnosis, two children were treated with steroid therapy after diagnosis, and 1 case of weakened immune function was used immunoglobulin for modulating immunity. All children improved and discharged, and no recurrence was observed until October 2021. Conclusion: In our study, the children with swollen lymph nodes in the neck were accompanied by tenderness and fever as the main clinical manifestations, without specificity. Histopathology and immunohistochemical examination by removing the affected lymph nodes could confirm HNL. Most children had a good prognosis and long-term follow-up were required.