Budd-Chiari syndrome and heparin-induced thrombocytopenia in polycythemia vera: Successful treatment with repeated TIPS and interferon alpha

Abstract

Polycythemia vera (PV) is a common cause of Budd-Chiari syndrome (BCS) and portal vein thrombosis (PVT). The postpartum period is a precipitating cofactor. An additional heparin-induced thrombocytopenia/thrombosis (HIT/T) leads to a life-threatening condition in which transjugular intrahepatic portosystemic shunting (TIPS) seems to be the only life-saving procedure. We describe the case of a subacute BCS and PVT in the late postpartum period. The diagnosis was established using CT scan, MRI, and Doppler ultrasonography of abdominal vessels and the laboratory findings were compatible with PV. After a successful creation of TIPS, a HIT/T worsened the hemorrhagic and thrombotic picture. TIPS procedure was successfully repeated and heparin was replaced with Fondaparinux and then vitamin K antagonist. The treatment with interferon alpha-2A, started after the normalization of liver functions, resulted in a complete remission within 6 months. The JAK2 V617F mutation clone remained undetectable after 2 years' follow-up.

Increased prevalence of heparin-induced thrombocytopenia in patients with Budd–Chiari syndrome: a retrospective analysis

“…As yet, there is no evidence to suggest the efficacy of dabigatran in this context. Fondaparinux and pegylated interferon α-2A have also been used successfully to treat HIT causing post-TIPS stent reocclusion in the context of acute BCS precipitated by pregnancy [27].…”

“…In a previous single-centre retrospective analysis studying 51 patients with BCS, the prevalence of HIT was found to be 14% [24], but there remains a dearth of studies specifically investigating the prevalence of HIT in patients with BCS. Currently, expert consensus through a series of case reports is the only existing evidence of HIT in BCS [9,23,[25][26][27][28]. To our knowledge, this is the first study to formalize this anecdotal evidence of HIT in BCS.…”

Portal vein thrombosis and Budd-Chiari syndrome as onset of polycythemia vera

“…Vice versa it will result in a rapid occlusion, and massive or sub-massive liver damage might appear as fulminant and unexpected hepatic failure. 11,[15][16][17] Therefore, at least four forms of Budd-Chiari syndrome can be identified: fulminant, acute, subacute, and chronic. Patients with fulminant Budd-Chiari syndrome usually present with jaundice associated with an increase in liver function enzymes, nausea, vomiting, abdominal pain, and, within eight weeks of the onset, hepatic encephalopathy.…”

“…Hepatomegaly is present in all four forms, while splenomegaly and esophageal varices are typical of subacute and chronic disease. 6,[15][16][17] Portal vein thrombosis and Budd-Chiari syndrome etiology can be considered to overlap, even if their clinical, laboratory and radiological profile might dif- fer. PVT usually involves the portal trunk extending, generally, toward the mesenteric vein and splenic vein.…”