Early medical treatment is life saving in acute Budd-Chiari due to polycythemia vera.
Here we describe two women with polycythemia vera presented with fulminant hepatic failure due to acute Budd-Chiari syndrome. Both had a history of severe abdominal pain and distention of short duration. Clinical and laboratory findings showed fulminant hepatic failure due to acute Budd-Chiari syndrome. Diagnosis was confirmed with abdominal ultrasonography and Doppler ultrasonography showing ascites, hepatomegaly, portal hypertension and total occlusion of hepatic veins. Complete blood count and other clinical findings were compatible with polycythemia vera in both patients. Patients were treated successfully with early administration of continuous heparin infusion, repeated phlebotomies and hydroxyurea. We emphasize here early diagnosis and effective treatment in such fulminant cases may be life saving.
Karadeniz Technical University, School of Medicine, Department of Internal Medicine, Division of Hematology, Trabzon, Turkey. samikarti@yahoo.com
Süleyman S
SS
Karti
Mustafa
M
Yilmaz
Polat
P
Kosucu
Elif
E
Altun
Jale
J
Kesen
Mehmet
M
Arslan
Orhan
O
Ozgur
Ercüment
E
Ovali
D000208:Acute Disease; D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use*; D006502:Budd-Chiari Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D004359:Drug Therapy, Combination; D005260:Female; D006493:Heparin / Q000627:therapeutic use*; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D017093:Liver Failure / Q000209:etiology; D011087:Polycythemia Vera / Q000150:complications*
D000925:Anticoagulants; D006493:Heparin; D006918:Hydroxyurea