OCCULT MYELOPROLIFERATIVE SYNDROME PRESEN TING WITH HEPATIC AND PORTAL-VEIN THROMBOSIS

In a 45-year-old woman presenting with subacute liver failure and port al hypertension the diagnostic workup revealed portal vein thrombosis and occlusion of small hepatic veins. An occult myeloproliferative syn drome was assumed. During full-dose heparin therapy the thrombotic pro cess progressed to segmental. venous small bowel infarctions, liver fa ilure and death. In-vitro culture of mononuclear blood cells showed sp ontaneous growth of erythroid precursor cells. Necropsy demonstrated a cute hemorrhagic necrosis of the liver, thrombotic material within the portal and mesenteric veins, thrombosis, dilatation, sclerosis, and p artial obliteration of small portal vein branches, and obliterative fi brosis and thrombosis of small intrahepatic veins. The bone marrow and spleen findings support the diagnosis of a myeloproliferative disorde r.