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. 2016:2016:2053257.
doi: 10.1155/2016/2053257. Epub 2016 Feb 22.

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

Vadim Gorodetskiy  1 , Wolfram Klapper  2 , Natalya Probatova  3 , Vladimir Vasilyev  1
Affiliations

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

Vadim Gorodetskiy et al. Case Rep Oncol Med. 2016.

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.

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Figures

Figure 1
Figure 1
Abdominal CT-scan.
Figure 2
Figure 2
Histological and immunohistochemical study.
See this image and copyright information in PMC

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