Polycythemia Vera with Budd-Chiari Syndrome

Abstract

Polycythaemia vera is a disorder of the hematopoietic stem cell which manifests as the overproduction of normal erythrocytes and variable excess production of WBC and platelets. It is a myeloproliferative disease, grouped with the Philadelphia chromosome-negative disorders and it’s very rare among children and teenagers. The thrombotic events are the most common complications due to hyperviscosity of the blood. The authors report the case of a 14-year-old girl who was admitted to the hospital for abdominal pain in the left quadrant and right hypochondrium. Workout findings reveal hepatosplenomegaly with infarction of the inferior pole of the spleen and hepatic vein thrombosis (specific with Budd-Chiari syndrome).