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Kaposiform haemangioendothelioma: clinical features, complications and risk factors for Kasabach–Merritt phenomenon
Author(s) -
Ji Y.,
Yang K.,
Peng S.,
Chen S.,
Xiang B.,
Xu Z.,
Li Y.,
Wang Q.,
Wang C.,
Xia C.,
Li L.,
Liu X.,
Lu G.,
Yang G.,
Wu H.
Publication year - 2018
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/bjd.16601
Subject(s) - medicine , interquartile range , lesion , odds ratio , confidence interval , cohort , univariate analysis , surgery , trunk , multivariate analysis , ecology , biology
Summary 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 KHE s. Among KHE s 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 KHE s appeared before 12 months of age. KHE s 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 .