title: Kaposiform lymphangiomatosis: unifying features of a heterogeneous disorder.
abstract: Kaposiform lymphangiomatosis (KLA) is a rare proliferation of abnormal lymphatic vessels often complicated by pleural/pericardial effusions and a consumptive coagulopathy that may lead to life threatening hemorrhage. Establishing the diagnosis is challenging due to the clinical heterogeneity and variable findings in laboratory values, radiographic features, and pathologic characteristics. We report three patients who had slowly progressive symptoms and presented with pleural or pericardial effusions, evidence of a consumptive coagulopathy and anemia. Despite being a rare and challenging diagnosis, KLA should be considered in patients presenting with non-specific indolent symptoms, pleural or pericardial effusions and laboratory evidence of a consumptive coagulopathy.
authors.affiliation: Center for Cancer and Blood Disorders, Children's National Health System, Washington, District of Columbia; Department of Pediatrics, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia.
authors.forename: Violet M
authors.initials: VM
authors.lastname: Fernandes
authors.forename: John H
authors.initials: JH
authors.lastname: Fargo
authors.forename: Surbhi
authors.initials: S
authors.lastname: Saini
authors.forename: Michael F
authors.initials: MF
authors.lastname: Guerrera
authors.forename: Leigh
authors.initials: L
authors.lastname: Marcus
authors.forename: Lori
authors.initials: L
authors.lastname: Luchtman-Jones
authors.forename: Denise
authors.initials: D
authors.lastname: Adams
authors.forename: Emily Riehm
authors.initials: ER
authors.lastname: Meier
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006390:Hemangioendothelioma / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D007223:Infant; D059885:Kasabach-Merritt Syndrome / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D064847:Multimodal Imaging; D010490:Pericardial Effusion / Q000473:pathology*; D010996:Pleural Effusion / Q000473:pathology*; D011379:Prognosis; D012514:Sarcoma, Kaposi / Q000175:diagnosis* / Q000628:therapy
keywords: GLA; KLA; consumptive coagulopathy; kaposiform lymphangiomatosis; pericardial effusion; pleural effusion