A 45-year-old lady presented with history of low grade fever, anorexia and left hypochondriac heaviness for 3 months with a history of melena 1 year ago. Physical examination revealed mild pallor, hepatomegaly of 3 cm below right costal margin which was firm, smooth and non-tender, and splenomegaly of 8 cm below left costal margin. There was no free fluid in abdomen. Hemoglobin was 10.9 g/dL, total leukocyte count (TLC) 6400/mm3, platelet count 154000/mm3, and prothrombin time was normal. Liver function tests revealed normal bilirubin with aspartate aminotransferase level (AST) of 50 IU/mL, alanine aminotransferase (ALT) of 67 IU/mL and alkaline phosphatase (ALP) of 1265 IU/mL with serum albumin of 4.16 g/dL. Upper gastrointestinal endoscopy (UGIE) showed 2 columns of grade 2 esophageal varices without red color signs, with large gastric varices with mild portal hypertensive gastropathy. N-butyl-2-cyanoacrylate glue was injected into the gastric varices after which melena subsided and subsequently the patient was lost to follow up. She presented again 1 year later with melena of 1 week duration. Low grade fever and anorexia were ongoing and during this period she had significant weight loss. She was admitted for evaluation. UGIE this time showed 2 columns of grade 2 esophageal varices with red color signs, with mild portal hypertensive gastropathy and hardened gastric varices without stigmata of recent bleed. Endoscopic variceal ligation was done. This time investigations showed hemoglobin of 9.9 g/dL, TLC 3700/mm3, platelet count of 96000/mm3, and normal prothrombin time. Bilirubin was normal with AST of 82 IU/mL, ALT of 74 IU/mL and alkaline phosphatase ALP of 1265 IU/mL with serum albumin of 3.9 g/dL. Calcium, phosphate and renal function tests were normal. Ultrasound showed mild hepatomegaly with heterogenous echotexture with large splenomegaly. Transient elastography revealed a liver stiffness of 21.5 KPa suggestive of cirrhosis. Hepatitis B surface antigen, anti-hepatitis C antibodies, autoimmune markers and Wilson disease work-up were negative. Triphasic contrast enhanced computed tomography (CT) abdomen showed enlarged heterogeneously enhancing liver with large spleen, both of which showed multiple small hypodense lesions, with multiple portal, gastrohepatic, and retroperitoneal lymph nodes. Contrast enhanced high resolution CT chest showed normal lung parenchyma with subcentimetric mediastinal lymph nodes. FNAC of portal lymph nodes was normal and culture did not grow any organism. Alpha feto-protein level was 1.33 ng/ml. Mantoux test was negative. Serum angiotensin converting enzyme level was 44 U/L (normal < 67 U/L).
Liver biopsy was done and histological findings are shown in Figure 1 and 2.
Figure 1.
Figure 2.
What is the diagnosis?
conflicts of interest
All authors have none to declare.
(Answer on the page 177)