Budd-Chiari syndrome in chronic myeloid leukemia.

A29-year-old Ph-positive patient in accelerated phase chronic myeloid leukemia (CML) with marked thrombocytosis developed fever, abdominal pain and signs of severe acute hepatitis, although was negative for viral markers. Real-time and Doppler ultrasound (US) scan showed marked hepatomegaly, caudate lobe hypertrophy (Figure 1), failure to visualize hepatic veins and their flow, portal vein ectasy with slow hepatopetal flow, splenomegaly and massive ascites. MRI and angiography confirmed occlusion of all hepatic veins and partial obstruction of the inferior vena cava hepatic segment, likely due to disproportionate caudate lobe enlargement. These findings suggested Budd-Chiari syndrome (BCS); treatment was diuretics, anticoagulants, chemotherapy and peritoneum-jugular shunt. Liver histology, obtained by uncomplicated percutaneous biopsy, confirmed the diagnosis (Figure 2)