Imatinib Treatment of Lymphangiomatosis (Generalized Lymphatic Anomaly)

• DOI: 10.6004/jnccn.2016.0045
• Publication Date: 2017-04-14T16:08:04Z
• Authors: Laura J. Libby; Navneet Narula; Helen Fernandes; James F. Gruden; David J. Wolf; Daniel M. Libby
• Keywords: Lymphangiomatosis; Chylothorax; Lymphangioleiomyomatosis; Imatinib Mesylate

Lymphangiomatosis (eg, generalized lymphatic anomaly) is an abnormal proliferation of endothelial cells. It often a childhood disease, but it may present in adulthood by infiltrating organs and cause obstruction, bleeding, or disruption flow. Pulmonary involvement be mild diffuse interstitial lung airway hemoptysis, chylothorax, chylopericardium, culminate respiratory failure. Treatment has been limited to surgical resection drainage procedures because there no accepted effective systemic therapy. This report presents patient with lymphangiomatosis life-threatening hemoptysis whom positive immunostaining for c-KIT suggested upregulation tyrosine kinase whose disease was controlled imatinib.