Primary isolated gastric varices in a patient with non hodgkins lymphoma

Abstract

A fifty-one year-old man admitted with dizziness after 2 weeks of bright red blood per rectum. He was undergoing CHOP chemotherapy for biopsy proven NHL. Review of systems: persistent left sided abdominal pain radiating to left flank and post-prandial fullness. EXAM remarkable for hepatosplenomegaly and heme-stools. Hct 30 MCV 77 Alb 3.5 AST 92 ALT 60 PT 14 PTT 30 CT: ill defined, 20cm splenic mass extending to the upper pole of left kidney, 19 cm liver, no focal lesions. EGD: esophagitis, grade II fundic varices Colonoscopy: hemorrhoids Doppler U/S: hepatopetal flow in the portal vein, patent splenic vein, persistent splenic mass. + HCV antibody. See figures: EGD, U/S, CT. IGV (isolated gastric varices) is a rare clinical entity typically seen after treatment of esophageal varices with sclerotherapy (secondary IGV). IGV is usually due to segmental portal hypertension. Medline lists 228 cases of segmental portal hypertension secondary to splenic vein thrombosis mostly associated with chronic pancreatitis, pancreatic cancer, and myeloproliferative disorders. Only 1 case of IGV in the setting of Hodgkin's lymphoma has been reported. IGV was found in only 4.7% cases in a review of 1,128 patients with portal hypertension. Of all IGV cases, only 15% had primary IGV as in our patient. Interestingly, our patient had no evidence of splenic vein obstruction as demonstrated by doppler U/S. IGV without venous thrombosis has been reported twice and the exact cause was not determined. We postulate that the presence of varices in this case, is due to extrinsic compression by the splenic mass. However, the rare chance of thrombus in proximal splenic vein cannot be excluded given the limitations of doppler U/S. There is a remote possibility of chronic HCV related liver disease contributing to fundic varices, however, occurrence of IGV in the setting of cirrhosis is less than 2% and there are no cases of IGV reported in the absence of cirrhosis. Though he is HCV antibody +, there is no indication of cirrhosis by the imaging studies noted above. In the absence of cirrhosis, HCV is unlikely to be the cause of his IGV. He remains asymptomatic for IGV. Liver bx is planned upon completion of CHOP. This is the first case of primary IGV in the setting of NHL. Absence of splenic vein thrombus as demonstrated by doppler U/S makes this case unique. Though rare, non thrombotic etiologies of portal hypertension should be considered in the differential of primary IGV.