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Combined Therapy with Rituximab Plus Cyclophosphamide/Vincristine/Prednisone for Sjogren’s Syndrome-Associated B-Cell Non-Hodgkin’s Lymphoma

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Abstract

Sjogren’s syndrome (SS) is characterized by an increased risk of developing non-Hodgkin’s lymphoma (NHL). Optimal treatment for NHL-complicating SS is not clearly established. NHL, which expresses the CD20 antigen on tumor cell surfaces, is a disease entity candidate to treatment with anti-CD20 monoclonal antibodies. We report clinical and immunological data of a patient with SS and NHL who was treated with a regimen consisting of cyclophosphamide/vincristine/prednisone (CVP) plus rituximab. A 68-year-old women had a 26-year history of SS and autoimmune thyroiditis. The clinical course of SS was complicated with severe splenomegaly. An increased percentage of CD19+ B cells (up to 30%) was detected in peripheral blood during follow-up. Clonal rearrangement of immunoglobulin heavy chain was detected. Low-grade B marginal zone lymphoma was diagnosed (peripheral blood immunophenotype: CD19 + CD20 + CD23 + sIg + Kappa; bone marrow immunophenotype: 25% lymphocytes; CD19 + CD20 + CD79A/BCL2+). She received a total of six cycles of CVP plus rituximab (375 mg/m2). Therapy was well tolerated, and B lymphocytes were depleted from the peripheral blood. Splenomegaly normalized. No evidence of neoplastic infiltration was detected in bone marrow after completion of therapy, while certain symptoms of SS (sicca and arthralgia) improved with treatment. CVP plus rituximab proved effective in a patient with SS with NHL.

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Acknowledgements

Juana Gil (Cellular Immunology, Immunology Department) performed peripheral blood immunophenotype analysis, Javier Menarguez (Pathology Department) assessed bone marrow biopsy analysis, Carmen Rodriguez-Sainz (Immunogenetics, Immunology Department) performed rearrangement of immunoglobulin heavy chain, and Margarita Rodríguez-Mahou (Autoimmunity Section, Immunology Department) performed autoantibody determinations. Juan Rodríguez-Molina performed humoral immunity studies.

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Authors and Affiliations

  1. Department of Immunology, University Hospital Gregorio Marañon, Dr Esquerdo 46, 28007, Madrid, Spain

    J. Carbone & E. Fernandez-Cruz

  2. Oncology Department, University Hospital Gregorio Marañon, Madrid, Spain

    R. Perez-Fernandez, A. Muñoz & P. Sabin

  3. Rheumatology Department, University Hospital Gregorio Marañon, Madrid, Spain

    L. Carreño

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  1. J. Carbone
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  2. R. Perez-Fernandez
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  3. A. Muñoz
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  5. L. Carreño
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Correspondence to J. Carbone.

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Carbone, J., Perez-Fernandez, R., Muñoz, A. et al. Combined Therapy with Rituximab Plus Cyclophosphamide/Vincristine/Prednisone for Sjogren’s Syndrome-Associated B-Cell Non-Hodgkin’s Lymphoma. Clinic Rev Allerg Immunol 34, 80–84 (2008). https://doi.org/10.1007/s12016-007-8025-2

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