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Updated: Sep 12, 2026
Whole-cell MALDI-TOF Mass Spectrometry is an Accurate and Rapid Method to Analyze Different Modes of Macrophage Activation
Published on: December 26, 2013
Macrophage activation syndrome in children with Kikuchi-Fujimoto disease
Zixuan Shen1, Jiayun Ling1, Xiaona Zhu1
1Department of Rheumatology and Immunology, Shenzhen Children's Hospital, 7019 Yitian Road, Shenzhen, 518038, China.
Background:
Kikuchi-Fujimoto disease (KFD) is typically a benign, self-limiting inflammatory disease. However, some patients may have a prolonged or recurrent disease course, or present with life-threatening complications such as macrophage activation syndrome (MAS). In this study, we aimed to describe the incidence and clinical features of MAS in KFD and to access potential laboratory markers for the diagnosis of KFD-associated MAS.
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. Data collected included clinical manifestations, laboratory and imaging findings, treatment, and clinical outcomes. Data were analyzed using GraphPad Prism 8.0 statistical software (GraphPad Software Inc., La Jolla, CA, USA). A receiver operating characteristic (ROC) curve analysis was further performed to access the potential predictors for the KFD-MAS diagnosis.
Results:
Of 58 patients with a histological diagnosis of KFD, 15 (25.9%) patients had MAS. Compared to patients without MAS, patients with KFD-MAS presented with a higher proportion of skin rash (26.7%, p = 0.01), glucocorticoid treatment (80%, p = 0.003), and disease recurrence (33.3%, p = 0.04). KFD-MAS patients had lower absolute peripheral white blood cell (WBC, p = 0.02), platelet (p = 0.002), serum albumin levels (p = 0.01), and lymphocyte count (p < 0.0001), and higher lactate dehydrogenase (LDH) levels (p < 0.0001). ROC curve analysis showed that the cutoff values of absolute lymphocyte count, an absolute platelet count, serum albumin level, and serum LDH level for KFD-MAS diagnosis were < 1235/μL, < 171 × 106/μL, < 35.6 g/L, and > 679 IU/mL, respectively.
Conclusions:
The presence of KFD-MAS in children may be more common than previously expected, especially in those with skin rash. KFD-MAS may be associated with a higher recurrence rate. An extremely elevated serum LDH level and moderate to severe lymphopenia may be useful diagnostic markers for MAS in KFD.
Trial Registration:
Not applicable; this was a retrospective study.
Insights
Macrophage activation syndrome (MAS) occurs in 25.9% of Kikuchi-Fujimoto disease (KFD) patients, often presenting with skin rash and higher recurrence rates. Elevated LDH and low lymphocyte counts may indicate KFD-MAS.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Hematology
Background:
- Kikuchi-Fujimoto disease (KFD) is typically a self-limiting inflammatory condition.
- However, KFD can present with severe complications like macrophage activation syndrome (MAS), necessitating further investigation into its incidence and diagnostic markers.
- This study focuses on KFD-associated MAS in pediatric patients.
Purpose of the Study:
- To determine the incidence and clinical characteristics of MAS in pediatric patients diagnosed with KFD.
- To identify potential laboratory markers for the early diagnosis of KFD-associated MAS.
Main Methods:
- Retrospective analysis of clinical data from 58 pediatric KFD patients (January 2015 - November 2021).
- Collected data included clinical manifestations, laboratory findings, imaging, treatment, and outcomes.
- Receiver operating characteristic (ROC) curve analysis was used to identify diagnostic predictors for KFD-MAS.
Main Results:
- 15 out of 58 KFD patients (25.9%) were diagnosed with MAS.
- KFD-MAS patients showed a higher incidence of skin rash (26.7%), glucocorticoid treatment (80%), and disease recurrence (33.3%).
- Key laboratory differences included lower WBC, platelet, albumin, and lymphocyte counts, and higher lactate dehydrogenase (LDH) levels in KFD-MAS patients.
Conclusions:
- KFD-MAS may be more prevalent in children than previously thought, particularly those with skin rash.
- MAS in KFD patients is associated with increased recurrence rates.
- Severely elevated serum LDH and significant lymphopenia are potential diagnostic indicators for MAS in KFD.
