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

Abstract EN

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.