Kaposiform lymphangiomatosis with Kasabach–Merritt phenomenon

Abstract

   Kaposiform lymphangiomatosis (KLA) is an aggressive lymphatic anomaly associated with bone involvement, serositis occurring at various sites, the development of Kasabach–Merritt phenomenon, and frequent infectious complications. The International Society for the Study of Vascular Anomalies classifies KLA as a subtype of generalized lymphatic anomaly. The mTOR-inhibitor rapamycin in combination with symptomatic treatment is the most common specific treatment. However, there are no standard approaches to the management of KLA. Even with modern diagnostic tools and combination therapy, the 5-year survival rate is 51 %, and the average life expectancy is 2.75 years. This article presents a classic case of KLA associated with Kasabach–Merritt phenomenon that was successfully managed with rapamycin and a liposomal form of doxorubicin as specific therapy. The patient's parents gave consent to the use of their child's data, including photographs, for research purposes and in publications.