SESSION TITLE: Critical Care Student/Resident Case Report Posters II
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: Polycythemia vera (PCV) is a unique myeloproliferative disorder (MPD) characterized by overproduction of erythrocytes and possibly granulocytes and platelets, a phenotype caused by the JAK2 mutation. PCV accounts for 10-40% of cases of Budd-Chiari syndrome (BCS), a rare condition resulting from obstruction of hepatic venous outflow. We present a critical care case of PCV with rapidly progressing disseminated thrombosis, initially presenting as BCS in a previously healthy patient.
CASE PRESENTATION: A 46-year-old male with no significant medical history presented with progressive abdominal distention, malaise and dyspnea on minimal exertion of three weeks duration. He described early satiety, diarrhea, orthopnea and decreased urination. He drank alcohol rarely, denied illicit substance use, chemical exposures and recent respiratory illness. He took no medications. Exam revealed a distressed hypotensive male who was alert and oriented. Other significant findings included abdominal distension, engorged superficial abdominal veins and leg edema. Laboratory tests showed leukocytosis, thrombocytosis and evidence of renal and liver failure. Toxicology and hepatitis panels were negative. Abdominal CT showed moderate ascites with SAAG 1.8, suggesting portal hypertension. Abdominal doppler US showed acute thrombosis of hepatic, portal and splenic veins. TPA was administered, followed by heparin and warfarin. Hypercoagulable workup later revealed JAK2 mutation, confirming PCV as underlying etiology for his BCS and disseminated thrombosis. Therapeutic phlebotomy and hydroxyurea were also initiated. Unfortunately, his hospital course was complicated by further thrombosis leading to a right parietal lobe infarction, disseminated intravascular coagulopathy, decompensated multi-organ failure and ultimately, death.
DISCUSSION: This case illustrates disseminated thrombosis, including Budd-Chiari syndrome from an aggressive myeloproliferative disorder with impressive hepatoportal tree thrombosis as the first manifestation of PCV. BCS treatment entails correction of liver functions and ascites by reducing hepatic congestion and preventing further thrombosis with anticoagulation. In some instances, invasive procedures such as TIPS, balloon dilation or stenting of hepatic veins are required. This patient’s extensive thrombosis, DIC and rapid decompensation made him a poor candidate for any invasive procedure.
CONCLUSIONS: This unusually rapid course of Jak2-positive MPD with extensive thrombosis of the portal tree, manifested many known complications of BCS as well as unusual manifestations of thrombosis with an eventual fatal outcome.
Reference #1: Menon KV, Shah V, Kamath P. The Budd-Chiari Syndrome. N Engl J Med. 2004;350:578-585.
Reference #2: Valla D, Casadevall N, Lacombe C, et al. Primary myeloproliferative disorder and hepatic vein thrombosis: a prospective study of erythroid colony formation in vitro in 20 patients with Budd-Chiari syndrome. Ann Intern Med. 1985;103(3):329-334.
DISCLOSURE: The following authors have nothing to disclose: Sheetal Gandotra, Gbolahan Ogunbayo, Siddharth Bansal, Akash Agarwal
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