A Case of Two Rarities: Budd-Chiari Syndrome and Primary Splenic Angiosarcoma

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Poster Presentation

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A previously well 55-year-old female presented to our ED with orange urine, recurrent fever, fatigue, poor appetite of about 3 weeks’ duration. Vital signs revealed fever of 38.5oC and tachycardia. Physical examination revealed an oriented, cachectic woman with bilateral pedal edema and a palpable left lobe of liver as well as fi rm, non-tender splenomegaly. Laboratory data included WBC of 15.8 x 103/μl (4.8-10.8 x 103/μL), hemoglobin of 7.4 g/dL (12.0-16.0 g/dL), platelet count of 112,000/μl (130-400,000/μl), prothrombin time of 13.4 secs (9.4-11.0 secs), INR of 1.3 (0.9-1.1), bilirubin of 2.2 mg/dL (0.2-1.1 mg/dL), albumin of 1.5 g/dL(3.4-4.6 g/dL), and AST and ALT of 33 mg/dl and 23 mg/dl respectively (5-38 mg/dl). CT abdomen and pelvis revealed calcifi ed IVC thrombus, chronic right hepatic infarcts, mild splenomegaly, moderate ascites and multiple small splenic lesions. MRI of abdomen and pelvis additionally reported patent portal vein and nonspecifi c small masses within the spleen, read as angiomas. Abdominal paracentesis showed SAAG >1.1 and negative cytology. A diagnosis of Primary Budd-Chiari syndrome was made. Extensive work up for hypercoagulable state and myeloproliferative disorders including bone marrow biopsy was unrevealing. Endoscopy revealed trace esophageal varices and normal colon. Patient was started on anticoagulation but was discontinued given progressive decline in platelet count to 25,000/μl. She progressed to acute fulminant hepatic failure over the next 10 days and was transferred to a liver transplant center where she underwent an orthotopic liver transplantation. Her post-operative course was complicated by severe thrombocytopenia, believed to be secondary to splenic sequestration, and a splenectomy was performed. Histopathology from her native liver and spleen unfortunately revealed primary splenic angiosarcoma. She subsequently died from respiratory complications 40 days aft er fi rst presenting. Budd-Chiari syndrome (BCS), a rare thrombotic occlusion of the hepatic venous outfl ow tract, was caused by an extremely rare and aggressive angiosarcoma of the spleen in our index case. With nonspecifi c radiologic features, the angiosarcoma was reported as multiple small lesions in the spleen with a broad diff erential. A thorough search for malignancy in the absence of thrombophilia disorder is crucial in BCS and unexplained fi ndings discovered during work up must be conclusively evaluated.

Publication Title

American Journal of Gastroenterology



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Presented at Reading Hospital Internal Medicine Research Day

Presented at the American College of Gastroenterology Annual Scientific Meeting
Las Vegas, NV

Open Access