Original ArticleKaposiform Hemangioendothelioma: Atypical Features and Risks of Kasabach-Merritt Phenomenon in 107 Referrals
Section snippets
Methods
We reviewed the medical records and database of the Vascular Anomalies Center at Children's Hospital Boston from 1991 to 2009 using the search terms “Kaposiform hemangioendothelioma,” “KHE,” “Kasabach-Merritt phenomenon,” “KMP,” “Kasabach-Merritt syndrome,” and “KMS” to define a cohort of patients with probable KHE. Our institutional review board approved this retrospective review. Our interdisciplinary team reviewed all cases and reached consensus on the diagnosis of KHE based on review of
Epidemiology
This KHE cohort represented referrals from 30 states and 15 countries. We assume that our center was involved in the vast majority of cases from Massachusetts. Given the estimated 1.4 million children <18 years old in Massachusetts in 200913 and 13 Massachusetts children with KHE in the same year, we estimate the prevalence of KHE as 0.91 case per 100 000 children. Over the past decade, there has been about one new case of KHE diagnosed in Massachusetts per year, yielding an incidence of 0.071
Discussion
KHE involves a spectrum of lesions from small, superficial tumors without KMP to large, infiltrative lesions with life-threatening complications including KMP. We have characterized the largest cohort of patients with KHE to date. Most patients have KMP and present in infancy with classic cutaneous lesions. Patients without cutaneous lesions present with atypical signs and symptoms and tend to be older. Numerous anatomic locations have been reported: cervicofacial region (sinuses,14, 15
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Supported by a Lovejoy Resident Research and Education Award and an American Society of Hematology Trainee Research grant (to S.C.) and by the National Institutes of Health/National Heart, Lung, and Blood Institute (K08 HL089509 to C.T.). The authors declare no conflicts of interest.