Primary Diffuse Large B-Cell Lymphoma of the Liver ina Patient with Sjogren Syndrome

Sj ̈ogren’s syndrome (SS) has the highest incidence of malignant lymphoproliferative disorders transformation among autoimmunediseases. We present a case of extranodal high grade lymphoma of the liver in a 52-year-old patient with long history of SS.Lymphoma manifested with sharp significant pain in the right hypochondrium, weakness, and profuse night sweats. Contrast-enhanced computed tomography scan (CT-scan) of the abdomen revealed multiple low density foci with homogeneous structureand clear contours in both lobes of the liver. Histologically, proliferation of medium sized lymphoma cells with round-oval andslightly irregular nuclei with fine chromatin was shown. Immunohistochemical and molecular features of the tumors alloweddiagnosis of diffuse large B-cell lymphoma (DLBCL). To exclude secondary liver lesion by non-Hodgkin lymphoma, chest andsmall pelvis CT-scan, endoscopy of upper and lower gastrointestinal tract and study of bone marrow were performed. After 8 cyclesof R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), the complete remission wasachieved, which persists after 45 months of follow-up. Primary hepatic lymphomas are extremely rare, and previously only low-grade hepatic lymphomas have been described in SS. To our knowledge, the patient described here represents the first reportedcaseofDLBCLwithprimaryliverinvolvementinSS.