Progression of nodal marginal zone lymphoma into diffuse large B cell lymphoma in a patient with Sjögren’s syndrome

Srpski Arhiv za Celokupno Lekarstvo

关键词: Sjögren’s syndrome;    nodal lymphoma of marginal zone;    immunohistochemistry;    diffuse large B cell lymphoma;   

DOI  :  10.2298/SARH1104229C

来源: DOAJ

【 摘 要 】

Introduction. Sjögren’s syndrome is a chronic autoimmune disorder carryingthe risk of the development of non-Hodgkin’s lymphoma, most frequentlymarginal zone lymphoma. Case Outline. A 66-year-old male patient withSjögren’s syndrome, after a year of the disease, developed a nodal marginalzone lymphoma with lymphoma cells in peripheral blood which had the followingimmunophenotype: CD19, CD20, CD22, CD19/kappa, CD79b+. After six cycles ofchemotherapy according to CHOP protocol (cyclophosphamide, doxorubicin,vincristine and prednisone) disease remission was achieved lasting fourmonths, followed by enlargement of lymph nodes in all areas (generalizedlymphadenopathy), splenomegaly and enlargement of the right parotid gland.Bone marrow biopsy and histology confirmed lymphoma of the same morphologicand immunohistochemic profile. Biopsy of a very enlarged hard right parotidgland, by using histology and immunohistochemistry, showed lymphoid tumourtissue with blast appearance and a number of nucleoli corresponding tocentroblasts and less to immunoblasts. Immunophenotypes of these cells wereas follows: CD79alfa+, CD20+, CD3-, bcl-2-; proliferative activity measuredwith KI-67 was high rating 60%. Histology and immunohistochemistry showed theco-existence of a diffuse large B cell lymphoma with marginal zone lymphoma.In spite of aggressive chemotherapy treatment according to protocol ESHAP(Vepesid 200 mg i.v. on 1st and 2nd day and 100 mg on 3rd, 4th and 5th day;Cisplatin 20-20-10 mg on 1st to 4th day) the disease showed a progressivecourse. Conclusion. In patients with Sjögren’s syndrome, the possibility oflymphoma should be kept in mind and in suspected cases timely diagnostic andtherapeutic measures should be undertaken.

【 授权许可】

Unknown