title: Primary Diffuse Large B-Cell Lymphoma of the Liver in a Patient with Sjogren Syndrome.
abstract: Sjögren's syndrome (SS) has the highest incidence of malignant lymphoproliferative disorders transformation among autoimmune diseases. 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 structure and clear contours in both lobes of the liver. Histologically, proliferation of medium sized lymphoma cells with round-oval and slightly irregular nuclei with fine chromatin was shown. Immunohistochemical and molecular features of the tumors allowed diagnosis of diffuse large B-cell lymphoma (DLBCL). To exclude secondary liver lesion by non-Hodgkin lymphoma, chest and small pelvis CT-scan, endoscopy of upper and lower gastrointestinal tract and study of bone marrow were performed. After 8 cycles of R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), the complete remission was achieved, 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 reported case of DLBCL with primary liver involvement in SS.
authors.affiliation: Department of Intensive Methods of Therapy, V.A. Nasonova Research Institute of Rheumatology, Russian Academy of Medical Sciences, Kashirskoye Shosse 34A, Moscow 115522, Russia.
authors.forename: Vadim
authors.initials: V
authors.lastname: Gorodetskiy
authors.affiliation: Department of Pathology, Hematopathology Section and Lymph Node Registry, Christian-Albrecht University Kiel and University Hospital Schleswig-Holstein, Arnold-Heller Strasse 3, 24105 Kiel, Germany.
authors.forename: Wolfram
authors.initials: W
authors.lastname: Klapper
authors.affiliation: Department of Pathology, N.N. Blokhin Russian Cancer Research Center, Russian Academy of Medical Sciences, Kashirskoye Shosse 24, Moscow 115478, Russia.
authors.forename: Natalya
authors.initials: N
authors.lastname: Probatova
authors.affiliation: Department of Intensive Methods of Therapy, V.A. Nasonova Research Institute of Rheumatology, Russian Academy of Medical Sciences, Kashirskoye Shosse 34A, Moscow 115522, Russia.
authors.forename: Vladimir
authors.initials: V
authors.lastname: Vasilyev