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 chil...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.展开更多
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 l...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.展开更多
Kikuchi-Fujimoto disease(KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon condition, typically characterized by lymphadenopathy and fevers. It usually has a benign course; however, it may prog...Kikuchi-Fujimoto disease(KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon condition, typically characterized by lymphadenopathy and fevers. It usually has a benign course; however, it may progress to fatality in extremely rare occasions. The diagnosis is made via lymph node biopsy and histopathology. Our patient was a young female who presented with shortness of breath, fever, and malaise. Physical examination revealed significant cervical and axillary lymphadenopathy. Chest X-ray displayed multilobar pneumonia. She required intubation and mechanical ventilation for progressive respiratory distress. Histopathology of lymph nodes demonstrated variable involvement of patchy areas of necrosis within the paracortex composed of karyorrhectic debris with abundant histiocytes consistent with KFD. After initial stabilization, the patient's condition quickly deteriorated with acute anemia, thrombocytopenia and elevated prothrombin time, partial prothrombin time, and D-dimer levels. Disseminated intravascular coagulopathy(DIC) ensued resulting in the patient's fatality. DIC in KFD is not well understood, but it is an important cause of mortality in patients with aggressive disease.展开更多
文摘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.
文摘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.
文摘Kikuchi-Fujimoto disease(KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon condition, typically characterized by lymphadenopathy and fevers. It usually has a benign course; however, it may progress to fatality in extremely rare occasions. The diagnosis is made via lymph node biopsy and histopathology. Our patient was a young female who presented with shortness of breath, fever, and malaise. Physical examination revealed significant cervical and axillary lymphadenopathy. Chest X-ray displayed multilobar pneumonia. She required intubation and mechanical ventilation for progressive respiratory distress. Histopathology of lymph nodes demonstrated variable involvement of patchy areas of necrosis within the paracortex composed of karyorrhectic debris with abundant histiocytes consistent with KFD. After initial stabilization, the patient's condition quickly deteriorated with acute anemia, thrombocytopenia and elevated prothrombin time, partial prothrombin time, and D-dimer levels. Disseminated intravascular coagulopathy(DIC) ensued resulting in the patient's fatality. DIC in KFD is not well understood, but it is an important cause of mortality in patients with aggressive disease.
