RESUMEN
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.
RESUMEN
Kikuchi-Fujimoto disease is a very rare disease, basically involving young adults and ubiquitously distributed. It is characterized by fever and benign lymph node swelling. The distinguishing features of this disease are cervical lymphadenopathy, constitutional symptoms resembling tuberculosis, and its penchant to affect young people of Oriental or Asian descent, especially women. We describe an instance of a 42-year-old female who arrived with multiple neck swellings. On physical examination, there was palpable right-sided cervical lymphadenopathy, while laboratory investigations were essentially within normal limits except for raised erythrocyte sedimentation rate and anemia. After cefepime and nonsteroidal anti-inflammatory medications were administered, symptoms subsided, and lymphadenopathy receded in the patient. This case supports the importance of histological evaluation to reach an exact diagnosis and guide treatment and the need to consider Kikuchi-Fujimoto disease in the differential diagnosis of lymphadenopathy.
RESUMEN
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.
RESUMEN
PURPOSE: Histiocytic necrotizing lymphadenitis (HNL) is an inflammatory disease of unknown etiology clinically characterized by painful lymphadenopathy. This study aimed to investigate the role of interferon (IFN)-α in the pathogenesis of HNL and the clinical significance of serum IFN-α levels for the diagnosis and monitoring of HNL disease activity. METHODS: This study enrolled 47 patients with HNL and 43 patients with other inflammatory diseases that require HNL differentiation including malignant lymphoma (ML), bacterial lymphadenitis, and Kawasaki disease. Expression of IFN-stimulated genes (ISGs) and MX1 in the lymph nodes was measured by real-time quantitative reverse transcription polymerase chain reaction and immunofluorescence staining, respectively. Enzyme-linked immunosorbent assay was used to quantify serum cytokine levels. The results were compared with the clinical features and disease course of HNL. RESULTS: Patients with HNL had a significantly elevated ISG expression in the lymph nodes compared with those with ML. MX1 and CD123, a specific marker of plasmacytoid dendritic cells (pDCs), were colocalized. In patients with HNL, serum IFN-α levels were significantly elevated and positively correlated with disease activity. The serum IFN-α level cutoff value for differentiating HNL from other diseases was 11.5 pg/mL. CONCLUSION: IFN-α overproduction from pDCs may play a critical role in HNL pathogenesis. The serum IFN-α level may be a valuable biomarker for the diagnosis and monitoring of disease activity in patients with HNL.
Asunto(s)
Células Dendríticas , Linfadenitis Necrotizante Histiocítica , Interferón-alfa , Ganglios Linfáticos , Humanos , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/sangre , Linfadenitis Necrotizante Histiocítica/inmunología , Masculino , Interferón-alfa/sangre , Femenino , Niño , Adolescente , Adulto , Células Dendríticas/inmunología , Células Dendríticas/metabolismo , Preescolar , Ganglios Linfáticos/patología , Proteínas de Resistencia a Mixovirus/genética , Proteínas de Resistencia a Mixovirus/metabolismo , Proteínas de Resistencia a Mixovirus/sangre , Adulto Joven , Persona de Mediana Edad , Linfoma/diagnóstico , Linfoma/inmunología , Linfoma/sangre , Síndrome Mucocutáneo Linfonodular/diagnóstico , Síndrome Mucocutáneo Linfonodular/inmunología , Síndrome Mucocutáneo Linfonodular/sangre , Biomarcadores/sangre , Citocinas/sangre , Citocinas/metabolismoRESUMEN
OBJECTIVES: To characterize the clinical features and to identify the predictors of recurrence of histiocytic necrotizing lymphadenitis (HNL) in Chinese children. STUDY DESIGN: This study retrospectively analyzed the clinical characteristics, laboratory and pathological findings, and recurrence status of children diagnosed with HNL at a single center in China from January 2018 to May 2023. Logistic regression analysis was employed to identify predictors of HNL recurrence. RESULTS: 181 Chinese children with histopathologically confirmed HNL were enrolled (121 males and 60 females). The mean age was 9.3 ± 2.9 years. The most prominent clinical features were fever (98.9%) and cervical lymphadenopathy (98.3%). Aseptic meningitis was the most frequent complication (38.5%), while hemophagocytic lymphohistiocytosis and autoimmune disease were rare (1.7% and 1.2%, respectively). Recurrence occurred in 12.7% of patients. Erythrocyte sedimentation rate (> 30 mm/h) was the significant predictors of HNL recurrence, with odds ratios of 6.107, respectively. CONCLUSION: Our study demonstrates that fever and cervical lymphadenopathy are the most frequent clinical manifestations of HNL in Chinese children, which often coexist with aseptic meningitis. HNL patients with risk factors require follow-up for recurrence.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Recurrencia , Humanos , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/epidemiología , Linfadenitis Necrotizante Histiocítica/patología , Masculino , Femenino , Niño , Estudios Retrospectivos , China/epidemiología , Linfadenopatía/etiología , Fiebre/etiología , Factores de Riesgo , Preescolar , Adolescente , Meningitis Aséptica/epidemiología , Meningitis Aséptica/diagnóstico , Pueblos del Este de AsiaRESUMEN
BACKGROUND: Cervical lymphadenopathy is common in children and has diverse causes varying from benign to malignant, their similar manifestations making differential diagnosis difficult. OBJECTIVE: This study aimed to investigate whether radiomic models using conventional magnetic resonance imaging (MRI) could classify pediatric cervical lymphadenopathy. METHODS: A total of 419 cervical lymph nodes from 146 patients, and encompassing four common etiologies (Kikuchi disease, reactive hyperplasia, suppurative lymphadenitis and malignancy), were randomly divided into training and testing sets in a ratio of 7:3. For each lymph node, 1,218 features were extracted from T2-weighted images. Then, the least absolute shrinkage and selection operator (LASSO) models were used to select the most relevant ones. Two models were built using a support vector machine classifier, one was to classify benign and malignant lymph nodes and the other further distinguished four different diseases. The performance was assessed by receiver operating characteristic curves and decision curve analysis. RESULTS: By LASSO, 20 features were selected to construct a model to distinguish benign and malignant lymph nodes, which achieved an area under the curve (AUC) of 0.89 and 0.80 in the training and testing sets, respectively. Sixteen features were selected to construct a model to distinguish four different cervical lymphadenopathies. For each etiology, Kikuchi disease, reactive hyperplasia, suppurative lymphadenitis, and malignancy, an AUC of 0.97, 0.91, 0.88, and 0.87 was achieved in the training set, and an AUC of 0.96, 0.80, 0.82, and 0.82 was achieved in the testing set, respectively. CONCLUSION: MRI-derived radiomic analysis provides a promising non-invasive approach for distinguishing causes of cervical lymphadenopathy in children.
Asunto(s)
Linfadenopatía , Imagen por Resonancia Magnética , Cuello , Humanos , Masculino , Femenino , Niño , Linfadenopatía/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Cuello/diagnóstico por imagen , Diagnóstico Diferencial , Preescolar , Adolescente , Estudios Retrospectivos , Lactante , Ganglios Linfáticos/diagnóstico por imagen , Ganglios Linfáticos/patología , RadiómicaRESUMEN
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign condition affecting young Oriental-Asian females. It is characterized by fever and tender cervical lymphadenopathy with an unclear aetiology, and in most longitudinal reviews, KFD occurs before systemic lupus erythematosus (SLE). Herein, the case of a 28-year-old Kuwaiti female without any relevant past medical history, who was simultaneously diagnosed with KFD and SLE following an Ebstein-Barr virus infection, is reported. The patient was treated with oral prednisolone, hydroxychloroquine, cyclosporin, and belimumab and her response was clinically and biochemically favourable. Although KFD is prevalent in Asian populations, it may affect all races. Early diagnosis of KFD is difficult, particularly when simultaneously diagnosed with SLE, but crucial to preventing inappropriate therapy. Clinicians need to know about this rare disease, especially when patients present with fever and swollen lymph nodes, due to a risk of misdiagnosis with tuberculosis or lymphoma, as these are more often thought to be the cause of such symptoms.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Lupus Eritematoso Sistémico , Adulto , Femenino , Humanos , Árabes , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Linfadenitis Necrotizante Histiocítica/patología , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Prednisolona/uso terapéutico , Prednisolona/administración & dosificación , KuwaitRESUMEN
Immune checkpoint inhibitors have become one of the effective means of solid tumor treatment, among which anti-programmed death-1 (PD-1) antibodies are more maturely applied and can effectively inhibit tumor immune escape, thus enhancing the anti-tumor effect, but it can also lead to a series of immune-related adverse events (irAEs) in the process of clinical use. Here, we report a Patient with pancreatic solid pseudopapilloma treated with Sintilimab for the fifteenth cycles who developed chills, fever, and lymph node enlargement. Considering that the patient did not have infection, without history of autoimmune disease, we diagnosed the patient with Sintilimab-induced histiocytic necrotizing lymphadenitis (Kikuchi disease). The symptoms are alleviated after rapid use of glucocorticoids. Histiocytic necrotizing lymphadenitis (Kikuchi lymphadenitis) with anti-programmed death-1 (PD-1) antibody is a rare immune-related adverse events (irAEs).
RESUMEN
Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disorder that usually presents with regional cervical lymphadenopathy and fever. We report a case of a 12-year-old female who complained of fever, night sweating, significant weight loss, and tender right cervical lymph node enlargement for 2 months. A full workup including laboratory tests and imaging studies, an excisional biopsy, and histopathological analysis were done, and the diagnosis of KFD was confirmed. The patient was treated with analgesia and oral prednisolone, resulting in good improvement. A high degree of clinical suspicion is imperative for physicians, given the rarity of the disease and the associated diagnostic challenges.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Linfadenopatía , Femenino , Humanos , Niño , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Linfadenopatía/complicaciones , Prednisolona/uso terapéutico , Fiebre/etiología , DolorRESUMEN
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare benign condition characterized by cervical lymphadenopathy and constitutional symptoms mimicking tuberculosis. We present the case of a 22-year-old male who presented with fever, dry cough, loss of appetite, multiple joint pains for 15 days, and loss of weight for one month. Physical examination revealed palpable cervical, occipital, axillary, and inguinal lymphadenopathy, and laboratory investigations were within normal limits except for raised erythrocyte sedimentation rate (ESR). Contrast-enhanced computed tomography (CECT) showed mediastinal lymphadenopathy with no pleuroparenchymal abnormality of the lung. Excision biopsy of a cervical lymph node confirmed necrotizing lymphadenitis consistent with KFD. The patient was treated with nonsteroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids, resulting in the resolution of symptoms and regression of lymphadenopathy. This case signifies the importance of considering KFD in the differential diagnosis of lymphadenopathy and highlights the significance of histopathological evaluation for accurate diagnosis and management guidance.
RESUMEN
Kikuchi-Fujimoto disease (KFD) is rare during pregnancy. It is characterized by necrotizing lymphadenitis and often occurs in young Asian women. We report a case of KFD during pregnancy, which was difficult to diagnose. A 37-year-old pregnant female (gestational week [GW] 7+5) was admitted to our hospital because of hyperemesis gravidarum. On the eighth day of hospitalization (GW 8+6), she suddenly developed a fever (38.0°C) with skin rash and posterior pharynx redness. Blood tests showed pancytopenia and abnormal liver function. The patient was misdiagnosed with severe Epstein-Barr virus infection and administered with prednisolone. Subsequently, cervical lymphadenopathy was observed, and biopsy results led to the diagnosis of KFD. Thereafter, her symptoms improved, and she was discharged at GW 13+4. KFD must be included as a differential diagnosis for patients with fever, abnormal liver function, and pancytopenia during pregnancy.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Humanos , Femenino , Linfadenitis Necrotizante Histiocítica/diagnóstico , Embarazo , Adulto , Complicaciones del Embarazo/diagnóstico , Hiperemesis Gravídica/diagnósticoRESUMEN
BACKGROUND: Histiocytic necrotizing lymphadenitis (HNL) is a self-limited inflammatory disease of unknown pathogenesis. A very small fraction of patients with HNL could develop hemophagocytic lymphohistiocytosis (HLH), a hyperinflammatory disorder. These patients are diagnosed as HNL with HLH (HNL-HLH). HNL-HLH in the pediatric population has been systemically studied, however, the clinical, laboratory, and radiological features and outcomes of adult patients with HNL-HLH remain to be explored. We aimed to explore the clinical, laboratory, and radiological features and outcomes of adult patients with HNL-HLH. METHODS: We collected the clinical data of patients with HNL-HLH admitted to the First Affiliated Hospital of Nanjing Medical University from October 2010 to June 2015. All the patients underwent lymph node biopsy and have a pathological diagnosis of HNL. The age, gender, clinical presentation, lymph node signs, laboratory findings and imaging data, and pathological findings of the patients were collected. RESULTS: In this study, we reported five adult patients with HNL-HLH. All five patients showed enlarged lymph nodes and prolonged fever. Laboratory findings were consistent with the diagnosis of HLH. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) showed enlarged lymph nodes with increased FDG uptake and splenic hypermetabolism could be present. All the patients responded well to corticosteroids and had a good prognosis. Two of the five patients were diagnosed with systemic lupus erythematosus during the follow-up. CONCLUSIONS: Our study demonstrated that adult patients with HNL-HLH showed distinct clinical, laboratory, and radiological features. And the prognosis is good and patients could be managed with steroids and supportive care.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Linfohistiocitosis Hemofagocítica , Adulto , Humanos , Niño , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/tratamiento farmacológico , Linfohistiocitosis Hemofagocítica/complicaciones , Linfohistiocitosis Hemofagocítica/diagnóstico , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Tomografía Computarizada por Tomografía de Emisión de Positrones/efectos adversos , Ganglios Linfáticos , Biopsia/efectos adversosRESUMEN
Histiocytic necrotizing lymphadenitis (HNL) is a benign, self-limiting disease that is rare clinically. The coexistence of HNL and tumor is rarer. We report a male patient who was preoperatively diagnosed with papillary thyroid carcinoma with cervical lymph nodes metastasis, and the postoperative pathological examination showed histiocytic necrotizing lymphadenitis combined with metastatic papillary thyroid carcinoma in the same single lymph node. More interestingly, EpsteinâBarr virus was positive in these lymph nodes by in situ hybridization. This may suggest a trigger for the coexistence of the two diseases.
Asunto(s)
Infecciones por Virus de Epstein-Barr , Linfadenitis Necrotizante Histiocítica , Neoplasias de la Tiroides , Humanos , Masculino , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/patología , Cáncer Papilar Tiroideo/patología , Infecciones por Virus de Epstein-Barr/patología , Herpesvirus Humano 4 , Ganglios Linfáticos/patología , Neoplasias de la Tiroides/complicaciones , Neoplasias de la Tiroides/patologíaRESUMEN
[Objective]To explore the etiology,pathogenesis and clinical treatment of histiocytic necrotizing lymphadenitis(HNL)based on the insidious pathogen warm disease theory.[Methods]To analyze the etiology,pathogenesis,characteristics of symptoms and transmission of the disease in Chinese medicine,and summarize the treatment principles based on the descriptions about insidious pathogen warm disease in ancient literature and modern researches on HNL,and cite a clinical case for verification.[Results]The pathogenesis of the HNL is mainly characterized by deficient healthy Qi leading to latent evil.The specific manifestation is that exogenous evils are latent in the Moyuan,which causes Yang Qi to be blocked and depressed and turns into heat.Evil heat spreads from the Moyuan to the Shaoyang tri-Jiao,leading to the generation of phlegm,static blood,turbid evil and toxin in the body,which in turn leads to disease.In terms of treatment,supporting healthy Qi and eliminating the evil is regarded as the law of treatment,and the emphasis is eliminating the evil.Aiming at the three pathogenetic links of latent evil,depressed heat and internal production of pathological products,the following treatment principles are formulated:expelling evils from Moyuan,dredging tri-Jiao and promoting the flow of Qi to make the evil heat go out,clearing away endogenous pathological products,removing toxin and dispersing knots.The focus of supporting healthy Qi is the protection of Qi and Yin as well as recuperation after recovery,which prevents evil Qi from remaining and causing the disease to reoccur.The medical case cited was a patient with HNL treated by applying the theory of insidious pathogen warm disease.Damp-heat and toxin brewing,phlegm combined with static blood was the traditional Chinese medicine(TCM)pattern of this case.The the prescriptions were based on Shengjiang Powder combined with Sanren Decoction,and Ganlu Xiaodu Pill successively,added and subtracted according to the syndrome,and the case achieved a significant effect.[Conclusion]The effect of treating HNL based on the insidious pathogen warm disease theory is quite good,which can provide new ideas and methods for the diagnosis and treatment of HNL.
RESUMEN
Kikuchi-Fujimoto disease (KFD), or Histiocytic Necrotizing Lymphadenitis, is a rare disease, with worldwide distribution but is best known in Japan and South Asia. The most common feature is cervical lymphadenopathy, accompanied by tenderness or high fever, with night sweats, but it can also be asymptomatic or with a very wide range of symptoms. The diagnosis is histopathological, on excisional biopsy. The Kikuchi-Fujmoto disease can mimic lymphoma but also tuberculosis and some autoimmune diseases, or be associated with them. Nephrologists need to be aware of it, considering the potential renal involvement. The association with systemic lupus erythematosus (SLE) is the most frequent but not the only one. Early diagnosis of this disease can prevent unnecessary investigations and aggressive therapies.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Lupus Eritematoso Sistémico , Humanos , Linfadenitis Necrotizante Histiocítica/complicaciones , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/patología , Diagnóstico Diferencial , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/patología , Diagnóstico Precoz , BiopsiaRESUMEN
Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a benign but rare disorder associated with febrile cervical lymphadenopathy in young adults. Here, we discuss a case of a young female patient presenting with left tender cervical lymphadenopathy that progressed bilaterally with a fever of unknown origin. Laboratory parameters showed persistent leukopenia, especially neutropenia, which fluctuated with the degree of symptom severity. Two months were taken to confirm the diagnosis of KFD based on the histological interpretation of the lymph node biopsy. Supportive management with analgesics and paracetamol formed the main treatment. This case highlights the challenges and importance of diagnosing KFD to exclude other serious conditions such as lymphoma, tuberculosis, or lupus lymphadenitis that share similar clinical manifestations as KFD.
RESUMEN
BACKGROUND: Kikuchi-Fujimoto disease (KFD) is typically a benign, self-limiting inflammatory disease. The diagnosis of KFD can be challenging for nonspecific symptoms, laboratory or imaging findings. In this study, we aimed to describe the clinical manifestations of patients with KFD and to access the potential role of serum cytokines in the diagnosis of this disease. METHODS: Patients with KFD were retrospectively enrolled from January 2015 to November 2021 at Shenzhen Children's Hospital. Clinical data were collected from inpatient or outpatient medical records. Serum cytokines were detected by the Flowcytomix technique. Serum levels of cytokines were compared between patients with KFD and SJIA, or patients with KFD and KD. The data of patients without MAS were further analyzed. A receiver operating characteristic (ROC) curve analysis was further performed to access the potential role of serum cytokines in the diagnosis of KFD. RESULTS: Serum cytokines were detected in 25 (43.8%, 25/57) patients with a histological diagnosis of KFD. Compared to SJIA or KD patients, the KFD group had a significantly higher IFN-γ/IL-6 ratio and much lower levels of serum IL-6. The median level of serum IFN-γ in KFD was 41.65 pg/ml (range, 21.04-70.74 pg/ml), which was much higher than that in SJIA (median: 3.33 pg/ml, p = 0.16) or KD (median: 2.6 pg/ml, p = 0.01). After excluding patients with MAS, there was statistical significance in all comparisons of serum IFN-γ, IFN-γ/IL-6 ratio, and serum IL-6. The cutoff values of serum IFN-γ, IL-6, and IFN-γ/IL-6 ratio for differentiating KFD from SJIA were > 8.48 pg/ml, < 47.42 pg/ml, and > 0.45, respectively. The cutoff values of serum IFN-γ, IL-6, and IFN-γ/IL-6 ratio for differentiating KFD from KD were > 8.56 pg/ml, < 50.45 pg/ml, and > 0.45, respectively. The specificity of all those cutoff values for differentiating KFD from SJIA or KD was ≥ 94.7%. CONCLUSIONS: For patients with fever of unknown etiology and lymphadenopathy, after excluding HLH or MAS, serum IFN-γ > 8.56 pg/mL and IFN-γ/IL-6 ratio > 0.45 may highly suggest the diagnosis of KFD; serum IL-6 > 50.45 pg/mL indicates that the probability of KFD may be small, and sJIA, KD, and acute infection should be excluded first.
Asunto(s)
Linfadenitis Necrotizante Histiocítica , Interferón gamma , Interleucina-6 , Humanos , Citocinas , Linfadenitis Necrotizante Histiocítica/diagnóstico , Pacientes Internos , Interleucina-6/sangre , Estudios Retrospectivos , Interferón gamma/sangreRESUMEN
INTRODUCTION: Myeloproliferative neoplasms (MPNs) are divided into BCR-ABL positive Chronic myeloid leukemia (CML) and BCR-ABL negative MPNs including Polycythemia vera (PV), Essential Thrombocythemia (ET) and Primary myelofibrosis (PMF). Evaluation of the Philadelphia chromosome in MPNs is a diagnostic requirement for classic CML. CASE REPORT: In 2020, a 37-year-old woman with negative cytogenetic testing for Janus kinase2 (JAK2), Calreticulin (CALR), myeloproliferative leukemia virus oncogene (MPL), and positive for BCR-ABL1 mutation with reticular fibrosis in bone marrow was diagnosed as CML. Some years ago, the patient had been diagnosed with PMF with evidence of histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease (KFD). The BCR-ABL fusion gene was initially evaluated which was negative. Then, Cutaneous squamous cell carcinoma (cSCC) was confirmed by Dermatopathologist with palpable splenomegaly and high white blood cell (WBC) count with basophilia. Finally, BCR-ABL was detected positive by the fluorescence in situ hybridization (FISH) and quantitative real-time polymerase chain reaction (qRT-PCR). In fact, the co-occurrence of PMF with CML was identified. CONCLUSION: This case study highlighted the importance of some cytogenetic methods in the detection and classification of MPNs. It is recommended that physicians pay more attention to it and be aware of the planning treatment.
RESUMEN
Kikuchi-Fujimoto disease, a rare form of necrotizing lymphadenitis, is an uncommon, benign, self-limiting disorder of obscure etiology. It affects mostly young adults of both genders. Clinically, it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss, splenomegaly, leucopenia, and elevated erythrocyte sedimentation rate feature in severely affected patients. Cutaneous involvement occurs in about 30%-40% of cases as facial erythema and nonspecific erythematous papules, plaques, acneiform or morbilliform lesions of great histologic heterogeneity. Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede, develop subsequently, or sometimes be associated concurrently with Kikuchi-Fujimoto disease. It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis, cat-scratch disease, Sweet's syndrome, Still's disease, drug eruptions, infectious mononucleosis, and viral or tubercular lymphadenitis are other common differentials. Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value. Since its diagnosis is exclusively from histopathology, it needs to be evaluated more carefully; an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials. Its treatment with systemic corticosteroids, hydroxychloroquine, or antimicrobial agents mostly remains empirical. The article reviews clinicoepidemiological, diagnostic, and management aspects of KFD from the perspective of practicing clinicians.