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Kaposiform haemangioendothelioma: clinical features, complications and risk factors for Kasabach-Merritt phenomenon
1Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, 610041, China.
Background:
Few studies have reported the clinical features, complications and predictors of Kasabach-Merritt phenomenon (KMP) associated with Kaposiform haemangioendothelioma (KHE).
Objectives:
To determine the clinical characteristics present at diagnosis and to identify features that may aid clinicians in managing KHE.
Methods:
We conducted a cohort study of 146 patients diagnosed with KHE.
Results:
KHE precursors or lesions were present at birth in 52·1% of patients. In 91·8% of patients, lesions developed within the first year of life. The median age at diagnosis of KHE was 2·3 months (interquartile range 1·0-6·0). The extremities were the dominant location, representing 50·7% of all KHEs. Among KHEs in the cohort, 63·0% were mixed lesions (cutaneous lesions with deep infiltration). Approximately 70% of patients showed KMP. A KHE diagnosis was delayed by ≥ 1 month in 65·7% of patients with KMP. Patients with KMP were more likely to have major complications than patients without KMP (P = 0·023). Young age (< 6 months), trunk location, large lesion size (> 5·0 cm) and mixed lesion type were associated with KMP in a univariate analysis. In the multivariate analysis, only age [odds ratio (OR) 11·9, 95% confidence interval (CI) 4·07-34·8; P < 0·001], large lesion size (OR 5·08, 95% CI 2·24-11·5; P < 0·001) and mixed lesion type (OR 2·96, 95% CI 1·23-7·13; P = 0·016) were associated with KMP.
Conclusions:
Most KHEs appeared before 12 months of age. KHEs are associated with various major complications, which can occur in combination and develop early in the disease process. Young age, large lesion size and mixed lesion type are important predictors of KMP.
Insights
Kaposiform hemangioendothelioma (KHE) often appears in infancy, with Kasabach-Merritt phenomenon (KMP) occurring in 70% of cases. Young age, large lesion size, and mixed lesion type predict KMP development and complications.
Area of Science:
- Vascular Anomalies
- Pediatric Oncology
- Hematology
Background:
- Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor with limited data on its clinical course.
- Kasabach-Merritt phenomenon (KMP) is a serious complication associated with KHE, necessitating further research into its predictors and clinical impact.
Purpose of the Study:
- To delineate the clinical characteristics of KHE at diagnosis.
- To identify key features predicting the occurrence of KMP in patients with KHE.
- To provide insights for improved clinical management of KHE.
Main Methods:
- A retrospective cohort study was conducted involving 146 patients diagnosed with KHE.
- Clinical data, including lesion presentation, KMP occurrence, and complications, were analyzed.
- Statistical analyses, including univariate and multivariate models, were employed to identify predictors of KMP.
Main Results:
- KHE lesions typically manifest within the first year of life, with a median diagnosis age of 2.3 months.
- Kasabach-Merritt phenomenon (KMP) was observed in approximately 70% of patients, often associated with delayed diagnosis and major complications.
- Univariate analysis identified young age, trunk location, large lesion size, and mixed lesion type as associated with KMP. Multivariate analysis confirmed young age (<6 months), large lesion size (>5 cm), and mixed lesion type as significant predictors.
Conclusions:
- Kaposiform hemangioendothelioma (KHE) predominantly affects infants, with a high incidence of Kasabach-Merritt phenomenon (KMP).
- KMP is linked to significant complications and is predicted by young age, large lesion size, and mixed lesion type.
- Early identification of these predictors is crucial for timely intervention and management of KHE-associated KMP.
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