[Portal vein and hepatic vein thrombosis in occult myeloproliferative syndrome. Progression of thrombosis under heparin therapy].
In a 45-year-old woman presenting with subacute liver failure and portal hypertension the diagnostic workup revealed portal vein thrombosis and occlusion of small hepatic veins. An occult myeloproliferative syndrome was assumed. During full-dose heparin therapy the thrombotic process progressed to segmental venous small bowel infarctions, liver failure and death. In-vitro culture of mononuclear blood cells showed spontaneous growth of erythroid precursor cells. Necropsy demonstrated acute hemorrhagic necrosis of the liver, thrombotic material within the portal and mesenteric veins, thrombosis, dilatation, sclerosis, and partial obliteration of small portal vein branches, and obliterative fibrosis and thrombosis of small intrahepatic veins. The bone marrow and spleen findings support the diagnosis of a myeloproliferative disorder.
Frey
H R
HR
Medizinische Abteilung, Kantonales Spital Sursee.
Suter
B
B
D017809:Fatal Outcome; D005260:Female; D006493:Heparin / Q000627:therapeutic use; D006503:Hepatic Veins; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D017093:Liver Failure / Q000209:etiology*; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000150:complications* / Q000175:diagnosis; D011169:Portal Vein; D013927:Thrombosis / Q000150:complications* / Q000188:drug therapy; D017211:Treatment Failure
D006493:Heparin