Primary hepatic extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type is associated with chronic inflammatory process

Primary hepatic extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type lymphoma is very rare disease. We reported two cases of primary hepatic MALT lymphoma associated with chronic inflammatory disorder. One case was a 74-year-old female complicated with asymptomatic primary biliary cirrhosis. Another case was a 73-year-old man presented with a mild hepatitis C. Both biopsy specimens showed diffuse infiltration of small atypical lymphoid cells that were phenotypically CD20+, CD79a+, CD5- and CD10-. Patients were received r-THP-COP (rituximab, pirarubicin, cyclophosphamide, vincristine, predonisolone) and achieved complete remission. Primary hepatic MALT lymphoma may be strongly associated with chronic inflammation, especially hepatitis C virus infection. However, the relationship between primary hepatic MALT lymphoma and PBC is still controversial, because the lymphoma complicated with PBC is extremity rare and there is no immunological and genetical study about it. We discuss the pathogenesis and clinical manifestation of this rare disease.