title: Management of disseminated intravascular coagulopathy with direct factor Xa inhibitor rivaroxaban in Klippel-Trénaunay syndrome.
abstract: Klippel-Trénaunay syndrome (KTS) is a rare congenital anomaly characterized by malformation of lymph and blood vessels as well as growth disturbance of soft tissue and bone. The clinical picture is variable and associated with an increased risk of thromboembolic events mediated by intravascular coagulopathy in venous malformations. Here, we report on a male patient with KTS suffering from recurrent deep vein thrombosis (DVT) and life-threatening bleeding due to consumptive coagulopathy. Furthermore, we describe the successful long-term anticoagulant management with rivaroxaban.
authors.affiliation: aDepartment of Internal Medicine, Division of Endocrinology, Diabetology, Vascular Medicine, Nephrology and Clinical Chemistry bDepartment of Internal Medicine, Division of Hematology and Oncology, Immunology, and Rheumatology, University of Tübingen, Tübingen, Germany.
authors.forename: Elko
authors.initials: E
authors.lastname: Randrianarisoa
authors.forename: Hans-Georg
authors.initials: HG
authors.lastname: Kopp
authors.forename: Bernd M
authors.initials: BM
authors.lastname: Balletshofer
authors.forename: Karl
authors.initials: K
authors.lastname: Jaschonek
authors.forename: Lothar
authors.initials: L
authors.lastname: Kanz
authors.forename: Hans-Ulrich
authors.initials: HU
authors.lastname: Haering
authors.forename: Kilian
authors.initials: K
authors.lastname: Rittig
mesh_terms: D000293:Adolescent; D000925:Anticoagulants / Q000008:administration & dosage*; D004211:Disseminated Intravascular Coagulation / Q000188:drug therapy* / Q000209:etiology; D015951:Factor Xa / Q000008:administration & dosage*; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications*; D008297:Male; D009025:Morpholines / Q000008:administration & dosage*; D000069552:Rivaroxaban; D013876:Thiophenes / Q000008:administration & dosage*
chemical_list: D000925:Anticoagulants; D009025:Morpholines; D013876:Thiophenes; D000069552:Rivaroxaban; D015951:Factor Xa