Hepatic MALTOMA in a Case of Chronic Hepatitis C

Abstract

Primary hepatic lymphoma (PHL) must be considered in any patient who presents with a liver mass or infiltration picture with cholestatic liver abnormality. The presence of constitutional symptoms and significant lymphadenopathy with Hepatitis C infection should invoke a search for PHL by liver biopsy (LB) as it is treatable. A 59-yr male presented with epigastric discomfort, fatigue, 30 lb. weight loss. Minimal alcohol use, 30-pack year smoking, no IVDU, blood transfusion but had a tattoo. He was afebrile, anicteric and had mild hepatomegaly with no lymphadenopathy. Laboratory work up showed elevated AST 112 U/L, ALT 333 U/L, with normal ALP 111 U/L, bilirubin 0.6 mg/dL, LDH 151 U/L, platelets 152,000/mm3, PT 11.1 sec, and INR 1.1. Iron studies, alpha 1 antitrypsin levels, ANA, AMA, and ASMA were normal. However, HCV was positive with HCV RNA of 2.3 million with genotype 2. Subsequent CT abdomen showed multiple enlarged lymph nodes within retroperitoneal and portacaval regions and a 1 cm hypo density in gall bladder (GB) wall. MRCP showed a contracted normal GB. LB confirmed mild chronic hepatitis, with lymphocytic portal inflammation with minimal fibrosis. EUS with biopsy confirmed benign intra-abdominal lymphadenopathy. Bone marrow biopsy showed no evidence of lymphoma. Immunohistochemistry on LB showed portal-based lymphocytic infiltrate with immunophenotype: CD20+ / BCL2+ / CD5- / CD10- / BCL6- / CD23- / cyclin D1-, with monoclonal pattern on immunoglobulin heavy chain gene rearrangement. A diagnosis of extra nodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) was made. We treated with sofosbuvir and ribavirin for 12 weeks and he achieved SVR 12. Repeat LB after 3 months after achieving SVR 12 confirmed reduced lymphocytic infiltration and our oncologist decided to observe him with no chemotherapy but his LFT's are normal and he was asymptomatic. PHL is a rare malignancy (0.016% of all non-Hodgkin lymphomas) and is often misdiagnosed. Among PHLs, low-grade marginal B-cell lymphoma (MZL) of MALT is extremely rare. Etiology of MZL is unclear HCV, HBV and EBV are implicated. Hepatic MZL affect middle-aged men and present with constitutional symptoms, hepatomegaly and cholestatic jaundice. LB compatible with lymphoma with no lymphoproliferative disease outside the liver is the presentation. Treating the primary infection and Chemotherapy with rituximab is standard.