A Sinister Cause of Hematemesis

Abstract

Question: A 52-year-old man with myelofibrosis presented to the emergency room with hematemesis. Earlier that day, he had experienced cough, rhinorrhea, and pharyngitis. On the evening of admission, he vomited blood, prompting him to seek medical attention. He denied fevers, chills, chest pain, abdominal pain, melena, hematochezia, prior history of upper gastrointestinal bleeding, nonsteroidal anti-inflammatory drug ingestion, or alcohol abuse. He was afebrile and hemodynamically stable. Physical examination was significant for splenomegaly and brown, guaiac-positive stool. His abdomen was nontender and nondistended without overt evidence of hepatomegaly. Pertinent laboratory results were as follows: blood urea nitrogen, 34 mg/dL; International Normalized Ratio, 1.3; white blood cell count 4.6 K/μL; platelets, 194 K/μL; and hemoglobin, 12.5 g/dL, which decreased to 9.6 g/dL on repeat 6 hours later. On presentation, he was given an intravenous bolus of esomeprazole 80 mg and initiated on an intravenous esomeprazole drip at 8 mg/h. An urgent upper endoscopy revealed isolated gastric varices with recent evidence of bleeding (Figure A, yellow arrow). He was given a bolus of intravenous octreotide 50 μg followed by an intravenous octreotide drip at 50 μg/h and ceftriaxone 1 g/d. The patient was transferred to the intensive care unit for further monitoring. What was the cause of this patient’s gastric varices (GV) and what is the next appropriate step? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. CT of the abdomen and pelvis revealed marked splenomegaly and prominent gastric (Figure B, C, arrow), paragastric, splenic, and mesenteric varices (Figure B, C, arrowhead). The patient underwent a liver biopsy, which revealed extramedullary hematopoiesis without evidence of fibrosis or nodular regenerative hyperplasia. Measurement of hepatic venous pressure gradient was 8 mmHg, confirming absence of right-sided portal hypertension. In our patient, sinistral portal hypertension and gastric variceal bleeding occurred secondary to splenomegaly from increased splenic blood flow owing to myelofibrosis-induced extramedullary hematopoiesis. Isolated GV are less prevalent than esophageal or gastroesophageal varices. Our patient had type 1 varices (IGV1), which are confined to the fundus. A common cause of IGV1 is splenic vein thrombosis and this diagnosis should be excluded. Initial management of gastric variceal bleed includes antibiotics, vasoactive drugs, and selective transfusion.1Garcia-Pagan J.C. Barrufet M. Cardenas A. et al.Management of gastric varices.Clin Gastroenterol Hepatol. 2014; 12: 919-928Abstract Full Text Full Text PDF PubMed Scopus (90) Google Scholar Beyond this initial management, studies have documented the use of splenectomy, splenic embolization, endoscopic variceal obturation (EVO) using tissue adhesives such as cyanoacrylate (CA), endoscopic injection sclerotherapy (EIS), variceal band ligation (EBL), transjugular intrahepatic portosystemic shunt and balloon-occluded retrograde transvenous obliteration.2Goh B.K. Chen J.J. Tan H.K. et al.Acute variceal bleed in a patient with idiopathic myelofibrosis successfully treated with endoscopic variceal band ligation.Dig Dis Sci. 2007; 52: 173-175Crossref PubMed Scopus (2) Google Scholar CA has demonstrated higher hemostasis and lower rebleeding rates compared with EBL and EIS. A recent study has shown that endoscopic ultrasonographic (EUS)-guided therapy for fundal varices with CA and coils may improve efficacy and decrease embolization of glue.3Binmoeller K.F. Weilert F. Shah J.N. et al.EUS-guided transesophageal treatment of gastric fundal varices with combined coiling and cyanoacrylate glue injection (with videos).Gastrointest Endosc. 2011; 74: 1019-1025Abstract Full Text Full Text PDF PubMed Scopus (145) Google Scholar CA is not currently available in the United States. However, many experts agree that, in patients with bleeding gastric fundal varices, the use of CA is preferred where available, with EBL as an alternative.1Garcia-Pagan J.C. Barrufet M. Cardenas A. et al.Management of gastric varices.Clin Gastroenterol Hepatol. 2014; 12: 919-928Abstract Full Text Full Text PDF PubMed Scopus (90) Google Scholar Splenectomy can be definitive treatment in patients with GV associated with sinistral portal hypertension.1Garcia-Pagan J.C. Barrufet M. Cardenas A. et al.Management of gastric varices.Clin Gastroenterol Hepatol. 2014; 12: 919-928Abstract Full Text Full Text PDF PubMed Scopus (90) Google Scholar In patients with myelofibrosis and splenomegaly, splenectomy is reserved for those with drug-refractory, symptomatic splenomegaly associated with frequent transfusions, portal hypertension, or severe thrombocytopenia. In our patient, splenectomy was chosen over EVO or transjugular intrahepatic portosystemic shunt owing to lack of available CA in the United States and the lack of elevated right-sided portal pressures. Postoperatively, his white blood cell count was 6.7 K/μL and platelet count was 279 K/μL. He received low-molecular-weight heparin for splenic and portal vein thrombosis, as well as hydrea and interferon. Repeat endoscopy 1 year after surgery showed no evidence of any varices. This case highlights the need for larger scale, randomized, controlled trials to guide management of gastric variceal bleeding. In addition, not all effective endoscopic and interventional techniques to treat GV are widely available, making it difficult for providers to follow current recommendations.