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Published on: September 14, 2010
Analysis of Risk Factors for Kasabach Merritt Phenomenom in Children With Kaposiform Hemangioendothelioma
Chen Chen1, Hanlei Yan2, Wei Yao1
1Department of Pediatric Surgery, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, China.
Background:
This study generalized and analyzed the clinical attributes observed in patients afflicted with Kaposiform hemangioendothelioma (KHE) with the aim of elucidating the risk factors contributing to the manifestation of Kasabach-Merritt phenomenon (KMP).
Methods:
We retrospectively analyzed 96 pediatric cases diagnosed with KHE at the Children's Hospital of Fudan University from January 2013 to December 2021. Among them, 62 patients (65%) showed KMP (KHE + KMP group), while 34 patients (35%) did not (KHE-KMP group). The risk factors for KMP associated with KHE were analyzed using univariate analysis and binary logistic regression analysis, comparing the differences between KHE + KMP group and KHE-KMP group.
Results:
Univariate analysis indicated no statistically significant differences between the two groups in gender, prematurity, family history, or color of involved skin. However, statistically significant differences were observed in age of onset, lesion site, and lesion depth. Multivariate analysis revealed significant associations: children with onset age ≤1 month had a 51-fold increased risk of KMP compared to those with onset age >1 month (95% CI 5.238-501.663); non-extremity lesion sites exhibited a 21-fold higher risk of KMP compared to extremity sites (95% CI 3.970-105.958); deeper lesions conferred a 5-fold higher risk of KMP compared to superficial lesions (95% CI 1.073-21.005); lesions >60 mm carried a 17-fold higher risk of KMP compared to lesions ≤60 mm (95% CI 2.999-96.157). A comprehensive predictive model was developed using the fitting formula: Logit (P) = 3.937∗(age at onset) + 1.558∗(lesion depth) + 3.021∗(lesion site) + 2.832∗(lesion size), demonstrating an accuracy of 82.9%. Furthermore, a scoring system was established to assess the likelihood of KMP occurrence. Children diagnosed with KHE were likely to have KMP if their score exceeded 72.5, as determined by Receiver Operating Characteristic (ROC) curve analysis.
Conclusion:
Age of onset ≤1 month, deeper lesions, non-extremity sites, and lesions ＞60 mm are independent risk factors for KMP in children with KHE. The cumulative presence of these factors escalates the likelihood of KMP development. Additionally, the identification of these factors allows for the early recognition of potential KMP cases among children with KHE, facilitating prompt therapeutic intervention.
Category Of The Manuscript:
Clinical Research article.
Level Of Evidence:
LEVEL Ⅲ.
Insights
Early onset (≤1 month), deep, large (>60 mm), non-extremity Kaposiiform hemangioendothelioma (KHE) lesions significantly increase Kasabach-Merritt phenomenon (KMP) risk in children. Identifying these factors aids early KMP recognition and intervention.
Area of Science:
- Pediatric Oncology
- Vascular Anomalies
- Hematology
Background:
- Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor in children.
- Kasabach-Merritt phenomenon (KMP) is a serious complication of KHE, involving thrombocytopenia and coagulopathy.
- Understanding KHE clinical attributes is crucial for identifying KMP risk factors.
Purpose of the Study:
- To analyze clinical features of KHE patients.
- To identify risk factors associated with KMP development in KHE.
- To develop a predictive model for KMP in pediatric KHE.
Main Methods:
- Retrospective analysis of 96 pediatric KHE cases (2013-2021).
- Comparison between KHE with KMP (n=62) and KHE without KMP (n=34) groups.
- Univariate and binary logistic regression analyses were performed to identify risk factors.
Main Results:
- Independent risk factors for KMP in KHE include: onset age ≤1 month (51x risk), non-extremity lesion site (21x risk), deeper lesions (5x risk), and lesion size >60 mm (17x risk).
- A predictive model with 82.9% accuracy was developed: Logit(P) = 3.937*(age) + 1.558*(depth) + 3.021*(site) + 2.832*(size).
- A scoring system indicated KMP likelihood if the score exceeded 72.5.
Conclusions:
- Early age of onset, deeper and larger lesions, and non-extremity locations are significant independent risk factors for KMP in pediatric KHE.
- The presence of multiple risk factors increases KMP likelihood.
- Early identification of these factors enables timely therapeutic intervention for KMP in KHE patients.
