Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by hemolytic anemia, hemoglobinuria, bone marrow failure, and hypercoagulability. Thrombosis is the leading cause of mortality and occurs in one-half of PNH patients, with the hepatic veins being the most common site. Patients with hepatic vein thrombosis (Budd-Chiari syndrome) can present with abdominal pain, hepatomegaly, jaundice, and ascites. Prognosis is poor for these patients; death may occur from liver failure, vessel rupture, intestinal ischemia, infarction, necrosis, or sepsis. The authors report three consecutive cases of successful treatment with catheter-directed thrombolysis and thrombectomy directly in the hepatic veins in patients with PNH who developed acute hepatic vein thrombosis. This treatment represents a potential bridge toward more curative therapies such as allogeneic bone marrow transplant.
References
Oct 1, 1985·Annals of Internal Medicine·P W Sholar, W R Bell
Jan 1, 1984·Clinical Neurology and Neurosurgery·S P Donhowe, R P Lazaro
Nov 1, 1983·AJR. American Journal of Roentgenology·L H GreenwoodG S Scoville
Feb 1, 1980·The American Journal of Medicine·D Zimmerman, W R Bell
Nov 9, 1995·The New England Journal of Medicine·P HillmenJ V Dacie
Jun 1, 1993·Journal of Hepatology·A OchsM Rössle
May 29, 2002·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Bernard G JaarMohamed G Atta
Oct 24, 2003·AJR. American Journal of Roentgenology·Michael F Goldberg, Hyun S Kim
Jun 17, 2004·British Journal of Haematology·Victor M MoyoRobert A Brodsky
Sep 24, 2004·Transplantation·Cristina RossiFranco Trevisani
Nov 5, 2004·Journal of Vascular and Interventional Radiology : JVIR·Jean-Pierre M CharonJonathan G Moss
Nov 24, 2004·American Journal of Hematology·Sambit K MohantyGurjeevan Garewal
Feb 15, 2005·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·Michal L MelamedMilagros D Samaniego
Jul 23, 2005·AJR. American Journal of Roentgenology·Claudio VignaliPasquale Petruzzi
Citations
Mar 26, 2009·International Journal of Hematology·Da-Long YinHong-Chi Jiang
Apr 18, 2009·Blood·Robert A Brodsky
Dec 3, 2011·Haematologica·David J AratenLucio Luzzatto
Oct 12, 2010·Gastroentérologie Clinique Et Biologique·J DumortierF Pilleul
Oct 5, 2010·Annals of Vascular Surgery·Xin-Hong HeHua-Qiao Tan
Sep 3, 2011·European Journal of Haematology·Alexander Röth, Ulrich Dührsen
Aug 12, 2009·Journal of Hepatology·Jildou HoekstraUNKNOWN European Network for Vascular Disorders of the Liver
Oct 18, 2016·Internal Medicine·Tomomi KogisoKatsutoshi Tokushige
May 19, 2017·Nature Reviews. Disease Primers·Anita HillRobert A Brodsky
Sep 2, 2018·European Journal of Haematology·Timothy DevosBérangère Devalet
Dec 15, 2012·Expert Review of Gastroenterology & Hepatology·Ross MacNicholasDhiraj Tripathi
May 16, 2020·Journal of Clinical Medicine·Siddhant Thukral, Suresh Vedantham
Mar 2, 2017·Therapeutic Advances in Hematology·Morag Griffin, Talha Munir
Jul 22, 2020·Digestive Diseases and Sciences·A SharmaC E Eapen
Apr 19, 2013·Experimental and Therapeutic Medicine·Ruihua WangXing Jin
Oct 19, 2013·Experimental and Therapeutic Medicine·Qingqiao ZhangBin Shen