Introduction. Sj?gren?s syndrome is a chronic autoimmune disorder carrying
the risk of the development of non-Hodgkin?s lymphoma, most frequently
marginal zone lymphoma. Case Outline. A 66-year-old male patient with
Sj?gren?s syndrome, after a year of the disease, developed a nodal marginal
zone lymphoma with lymphoma cells in peripheral blood which had the following
immunophenotype: CD19, CD20, CD22, CD19/kappa, CD79b+. After six cycles of
chemotherapy according to CHOP protocol (cyclophosphamide, doxorubicin,
vincristine and prednisone) disease remission was achieved lasting four
months, followed by enlargement of lymph nodes in all areas (generalized
lymphadenopathy), splenomegaly and enlargement of the right parotid gland.
Bone marrow biopsy and histology confirmed lymphoma of the same morphologic
and immunohistochemic profile. Biopsy of a very enlarged hard right parotid
gland, by using histology and immunohistochemistry, showed lymphoid tumour
tissue with blast appearance and a number of nucleoli corresponding to
centroblasts and less to immunoblasts. Immunophenotypes of these cells were
as follows: CD79alfa+, CD20+, CD3-, bcl-2-; proliferative activity measured
with KI-67 was high rating 60%. Histology and immunohistochemistry showed the
co-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 progressive
course. Conclusion. In patients with Sj?gren?s syndrome, the possibility of
lymphoma should be kept in mind and in suspected cases timely diagnostic and
therapeutic measures should be undertaken.
Molecular characteristics of diffuse large B-cell lymphoma in human immunodeficiency virus-infected and -uninfected patients in the pre-highly active antiretroviral therapy and pre-rituximab era
