title: Imatinib Treatment of Lymphangiomatosis (Generalized Lymphatic Anomaly).
abstract: Lymphangiomatosis (eg, generalized lymphatic anomaly) is an abnormal proliferation of lymphatic endothelial cells. It is often a childhood disease, but it may present in adulthood by infiltrating organs and cause obstruction, bleeding, or disruption of lymphatic flow. Pulmonary involvement may be mild or cause diffuse interstitial lung disease, airway obstruction, hemoptysis, chylothorax, chylopericardium, and culminate in respiratory failure. Treatment has been limited to surgical resection or drainage procedures because there is no accepted effective systemic therapy. This report presents a patient with lymphangiomatosis and life-threatening hemoptysis in whom positive immunostaining forc-KITsuggested upregulation of tyrosine kinase and whose disease was controlled with imatinib.
authors.affiliation: Department of Medicine, Division of Pulmonary and Critical Care Medicine, Joan and Sanford I. Weill Medical College of Cornell University and The New York-Presbyterian Hospital Weill Cornell Medical Center, New York, New York
authors.forename: Laura J
authors.initials: LJ
authors.lastname: Libby
authors.affiliation: Department of Pathology and Laboratory Medicine, Joan and Sanford I. Weill Medical College of Cornell University and The New York-Presbyterian Hospital Weill Cornell Medical Center, New York, New York
authors.forename: Navneet
authors.initials: N
authors.lastname: Narula
authors.affiliation: Department of Pathology and Laboratory Medicine, Joan and Sanford I. Weill Medical College of Cornell University and The New York-Presbyterian Hospital Weill Cornell Medical Center, New York, New York
authors.forename: Helen
authors.initials: H
authors.lastname: Fernandes
authors.affiliation: Department of Radiology, Division of Body Imaging, Joan and Sanford I. Weill Medical College of Cornell University and The New York-Presbyterian Hospital Weill Cornell Medical Center, New York, New York
authors.forename: James F
authors.initials: JF
authors.lastname: Gruden
authors.affiliation: Department of Medicine, Division of Hematology and Oncology, Joan and Sanford I. Weill Medical College of Cornell University and The New York-Presbyterian Hospital Weill Cornell Medical Center, New York, New York
authors.forename: David J
authors.initials: DJ
authors.lastname: Wolf
authors.affiliation: Department of Medicine, Division of Pulmonary and Critical Care Medicine, Joan and Sanford I. Weill Medical College of Cornell University and The New York-Presbyterian Hospital Weill Cornell Medical Center, New York, New York
authors.forename: Daniel M
authors.initials: DM
authors.lastname: Libby
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001706:Biopsy; D004252:DNA Mutational Analysis; D005260:Female; D006801:Humans; D000068877:Imatinib Mesylate / Q000627:therapeutic use*; D008202:Lymphangioma / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D008875:Middle Aged; D000072078:Positron Emission Tomography Computed Tomography; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D019233:Retreatment; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D047428:Protein Kinase Inhibitors; D000068877:Imatinib Mesylate