Abstract

Hemophagocytic syndrome (HPS) is a rare disease in clinical practice, and there are often cases of delayed diagnosis. At present, researchers have applied 18F-FDG PET/CT in the differential diagnosis of HPS, but no consensus has been formed. Therefore, this study aims to systematically evaluate the application value of 18F-FDG PET/CT in the diagnosis of HPS patients. PubMed, Embase, Cochrane Library, Chinese National Knowledge Infrastructure (CNKI), Wangfang database (Wangfang), and Chinese Biomedical Network (CBM) were searched to collect the relevant studies of 18F-FDG PET/CT in the diagnosis of HPS. Data from the articles were screened and extracted for meta-analysis using Stata16.0 software. A total of 10 retrospective studies, including 300 patients, were included in this meta-analysis. The meta-analysis results showed that the pooled sensitivity was 0.82 (95% CI: 0.67–0.95), specificity was 0.72 (95% CI: 0.51–0.86), positive likelihood ratio was 2.89 (95% CI: 1.46–5.75), positive likelihood ratio was 0.25 (95% CI: 0.12–0.54), diagnostic odds ratio was 2.89 (95% CI: 1.46–5.75), and AUC was 0.84 (95% CI: 0.81–0.87). The SUVmax in the liver, spleen, lymph nodes, and bone marrow of HPS patients was greater than 2.5, and the SUVmax in the spleen, lymph nodes, and bone marrow of malignant HPS patients was higher than that of benign HPS patients. The difference was statistically significant (P < 0.05). According to the existing literature evidence, 18F-FDG PET/CT is an effective method for diagnosing HPS.

Highlights

  • Hemophagocytic syndrome (HPS), known as hemophagocytic lymph hyperplasia, is a clinical illness in which excessive inflammatory responses are induced by primary or secondary immune system disorders (HLH)

  • E conclusion in previous studies was controversial, and the sample size was small. e meta-analysis could pool the studies of small sample size, and we could draw a stable conclusion by means of meta-analysis. is is the first metaanalysis to study the diagnosis of hemophagocytic syndrome by the 18F-FDG PET/CT [9, 17–25]. e ten studies included in this meta-analysis [26–28] were all relevant reports on the use of 18F-FDG PET/CT for the differential or diagnosis of HPS. e conclusions of the above 10 studies were conflict; the objective of the study was to evaluate the diagnostic performance of 18F-FDG PET/CT for the hemophagocytic syndrome

  • After SROC curve analysis of the data of the seven included articles, the results showed that the Area Under Curve (AUC) was 0.84, suggesting a high accuracy of 18F-FDG PET/CT in the differential diagnosis of benign and malignant hemophagocytic syndrome

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Summary

Introduction

Hemophagocytic syndrome (HPS), known as hemophagocytic lymph hyperplasia, is a clinical illness in which excessive inflammatory responses are induced by primary or secondary immune system disorders (HLH). The clinical manifestations of HPS are diverse and lack specificity and usually mimic or overlap with the clinical manifestations of diseases such as systemic inflammatory response syndrome, multiple organ failure, and sepsis [6]. HPS mainly includes two types: primary and secondary. Primary HPS is caused by hereditary immune dysfunction, is known as familial hemophagocytic lymphohistiocytosis, and primarily occurs in infants [7]. Secondary HPS is mainly caused by malignant tumors, autoimmune diseases, and chronic viral infections and is less likely to be secondary to conditions such as chronic diseases (chronic nephritis, liver disease, diabetes, and chronic granulomatous diseases) and pernicious anemia [8–10]. Among the causes of secondary HPS, malignant tumors are the most common (about 45%), and most of them are hematological

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