Histiocytic necrotizing lymphadenitis with hemophagocytic lymphohistiocytosis in adults : A single-center analysis of 5 cases
© 2024 The Authors. Immunity, Inflammation and Disease published by John Wiley & Sons Ltd..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:12 |
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| Enthalten in: |
Immunity, inflammation and disease - 12(2024), 2 vom: 27. Feb., Seite e1202 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Chen, Qingqing [VerfasserIn] |
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| Links: |
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| Themen: |
Hemophagocytic lymphohistiocytosis |
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| Anmerkungen: |
Date Completed 28.02.2024 Date Revised 29.02.2024 published: Print Citation Status MEDLINE |
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| doi: |
10.1002/iid3.1202 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM369012550 |
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| 245 | 1 | 0 | |a Histiocytic necrotizing lymphadenitis with hemophagocytic lymphohistiocytosis in adults |b A single-center analysis of 5 cases |
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| 520 | |a © 2024 The Authors. Immunity, Inflammation and Disease published by John Wiley & Sons Ltd. | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Kikuchi-Fujimoto disease | |
| 650 | 4 | |a hemophagocytic lymphohistiocytosis | |
| 650 | 4 | |a histiocytic necrotizing lymphadenitis | |
| 700 | 1 | |a Zhang, Jing |e verfasserin |4 aut | |
| 700 | 1 | |a Huang, Huijun |e verfasserin |4 aut | |
| 700 | 1 | |a Qiu, Tonglu |e verfasserin |4 aut | |
| 700 | 1 | |a Jin, Ze |e verfasserin |4 aut | |
| 700 | 1 | |a Shi, Yu |e verfasserin |4 aut | |
| 700 | 1 | |a Zhu, Huayuan |e verfasserin |4 aut | |
| 700 | 1 | |a Fan, Lei |e verfasserin |4 aut | |
| 700 | 1 | |a Li, Jianyong |e verfasserin |4 aut | |
| 700 | 1 | |a Shi, Wenyu |e verfasserin |4 aut | |
| 700 | 1 | |a Miao, Yi |e verfasserin |4 aut | |
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