Budd-Chiari syndrome and heparin-induced thrombocytopenia in polycythemia vera: successful treatment with repeated TIPS and interferon alpha.
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.
Akoum
Riad
R
Department of Oncology, Rizk Hospital, Beirut, Lebanon.
Mahfoud
Daniel
D
Ghaoui
Albert
A
Haddad
Nadine
N
Mahfoud
Nathalie
N
Farhat
Hussein
H
Khoury
Joe
J
D000328:Adult; D000925:Anticoagulants / Q000009:adverse effects; D006502:Budd-Chiari Syndrome / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D000077425:Fondaparinux; D006493:Heparin / Q000009:adverse effects; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D016898:Interferon-alpha / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D011087:Polycythemia Vera / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D011134:Polysaccharides / Q000627:therapeutic use; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D013921:Thrombocytopenia / Q000139:chemically induced / Q000150:complications / Q000628:therapy*; D014057:Tomography, X-Ray Computed; D018608:Ultrasonography, Doppler; D014812:Vitamin K / Q000819:agonists
D000925:Anticoagulants; D007155:Immunologic Factors; D016898:Interferon-alpha; D011134:Polysaccharides; D014812:Vitamin K; D006493:Heparin; D000077425:Fondaparinux