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 521% of patients. In
918% of patients, lesions developed within the first year of life. The median age
at diagnosis of KHE was 23 months (interquartile range 10–60). The extremities
were the dominant location, representing 507% of all KHEs. Among KHEs in the
cohort, 630% were mixed lesions (cutaneous lesions with deep infiltration).
Approximately 70% of patients showed KMP. A KHE diagnosis was delayed by ≥
1 month in 657% of patients with KMP. Patients with KMP were more likely to
have major complications than patients without KMP ( P=0023). Young age (
6 months), trunk location, large lesion size (> 50 cm) and mixed lesion type
were associated with KMP in a univariate analysis. In the multivariate analysis, only
age [odds ratio (OR) 119, 95% confidence interval (CI) 407–348; P
large lesion size (OR 508, 95% CI 224–115; P
(OR 296, 95% CI 123–713; P=0016) 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.</p