Abstract

INTRODUCTION: Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by excessive platelet production leading to a prothrombotic state. Thrombosis of the portal venous system has been well-established in patients with ET and can cause life-threatening complications in certain situations. In pregnant females, ET alone has been associated with higher rates of fetal demise and treatment options for acute complications may be limited. We report a unique case in which a 28-week pregnant female with ET complicated by splenic thrombosis was successfully treated for life-threatening bleeding gastric varices. CASE DESCRIPTION/METHODS: A 28-week pregnant, 38-year-old female with a previous history of obstetric complications (intrauterine growth restriction, HELLP syndrome) requiring preterm C-sections, and ET complicated by splenic vein thrombosis and marked splenomegaly presented with hematemesis and melena. Upon admission, she was found to have a hemoglobin of 7.5g/dL (baseline 13g/dL pre-pregnancy). Following stabilization, an urgent EGD was performed noting large, non-bleeding gastric varices within the gastric cardia and fundus with stigmata of recent bleeding (Figure 1). Follow-up magnetic resonance venography confirmed presence of current splenic vein thrombosis. To prevent recurrent bleeding, and possible obstetric complications, an empiric splenic artery embolization followed by open splenectomy was performed. No complications were encountered in either the mother or baby. Post-operatively, her hemoglobin stabilized, and she was discharged in stable condition. DISCUSSION: Abdominal thrombotic complications in ET has been well established. Thrombosis of the portal venous system, in-particular the splenic vein, can lead to the formation of gastric varices which can cause life-threatening bleeding. Splenectomy has been referred as the treatment of choice in these situations however its role during pregnancy has not been well-reported. We report a successful case in which an open splenectomy was safely performed in a 28-week pregnant patient for the treatment of bleeding gastric varices.Figure 1.: Large gastric varices within the gastric fundus and cardia on retroflexed view.

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