Primary Diffuse Large B-Cell Lymphoma of the Liver in a Patient with Sjogren Syndrome
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...
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| Format: | Article |
| Language: | English |
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Wiley
2016-01-01
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| Series: | Case Reports in Oncological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2016/2053257 |
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| author | Vadim Gorodetskiy Wolfram Klapper Natalya Probatova Vladimir Vasilyev |
| author_facet | Vadim Gorodetskiy Wolfram Klapper Natalya Probatova Vladimir Vasilyev |
| author_sort | Vadim Gorodetskiy |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-8c93d9e497924ea6acd3b5fd91ad4734 |
| institution | Kabale University |
| issn | 2090-6706 2090-6714 |
| language | English |
| publishDate | 2016-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Oncological Medicine |
| spelling | doaj-art-8c93d9e497924ea6acd3b5fd91ad47342025-02-03T01:30:14ZengWileyCase Reports in Oncological Medicine2090-67062090-67142016-01-01201610.1155/2016/20532572053257Primary Diffuse Large B-Cell Lymphoma of the Liver in a Patient with Sjogren SyndromeVadim Gorodetskiy0Wolfram Klapper1Natalya Probatova2Vladimir Vasilyev3Department of Intensive Methods of Therapy, V.A. Nasonova Research Institute of Rheumatology, Russian Academy of Medical Sciences, Kashirskoye Shosse 34A, Moscow 115522, RussiaDepartment of Pathology, Hematopathology Section and Lymph Node Registry, Christian-Albrecht University Kiel and University Hospital Schleswig-Holstein, Arnold-Heller Strasse 3, 24105 Kiel, GermanyDepartment of Pathology, N.N. Blokhin Russian Cancer Research Center, Russian Academy of Medical Sciences, Kashirskoye Shosse 24, Moscow 115478, RussiaDepartment of Intensive Methods of Therapy, V.A. Nasonova Research Institute of Rheumatology, Russian Academy of Medical Sciences, Kashirskoye Shosse 34A, Moscow 115522, RussiaSjögren’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.http://dx.doi.org/10.1155/2016/2053257 |
| spellingShingle | Vadim Gorodetskiy Wolfram Klapper Natalya Probatova Vladimir Vasilyev Primary Diffuse Large B-Cell Lymphoma of the Liver in a Patient with Sjogren Syndrome Case Reports in Oncological Medicine |
| title | Primary Diffuse Large B-Cell Lymphoma of the Liver in a Patient with Sjogren Syndrome |
| title_full | Primary Diffuse Large B-Cell Lymphoma of the Liver in a Patient with Sjogren Syndrome |
| title_fullStr | Primary Diffuse Large B-Cell Lymphoma of the Liver in a Patient with Sjogren Syndrome |
| title_full_unstemmed | Primary Diffuse Large B-Cell Lymphoma of the Liver in a Patient with Sjogren Syndrome |
| title_short | Primary Diffuse Large B-Cell Lymphoma of the Liver in a Patient with Sjogren Syndrome |
| title_sort | primary diffuse large b cell lymphoma of the liver in a patient with sjogren syndrome |
| url | http://dx.doi.org/10.1155/2016/2053257 |
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