Article
Kaposiform lymphangiomatosis effectively treated with MEK inhibition.
EMBO molecular medicine - 7 Oct 2020
Foster Jessica B, Li Dong, March Michael E, Sheppard Sarah E, Adams Denise M, Hakonarson Hakon, Dori Yoav
Abstract excerpt
Kaposiform lymphangiomatosis (KLA) is a rare lymphatic anomaly primarily affecting the mediastinum with high mortality rate. We present a patient with KLA and significant disease burden harboring a somatic point mutation in the Casitas B lineage lymphoma (CBL) gene. She was treated with MEK inhibition with complete resolution of symptoms, near-complete resolution of lymphatic fluid burden, and remodeling of her...
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