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Long-term remission of an EBV-positive B cell lymphoproliferative disorder associated with rheumatoid arthritis under methotrexate with anti-CD20 monoclonal antibody (Rituximab) monotherapy

Leukemia volume 16, pages 2173–2174 (2002)Cite this article

After the first report by Kamel et al1 in 1993 about rheumatoid arthritis (RA)-associated lymphomas regressing after withdrawal of methotrexate, there is increasing concern about the role of methotrexate (MTX) in developing such malignancies. A particular group of RA-associated lymphomas may share common characteristics with post-transplant lymphoproliferative disorders (PT-LPD).2 We report the case of a fulminant polyclonal B cell, EBV-positive lymphoproliferative disorder occurring in a patient with juvenile rheumatoid arthritis under MTX. Long-term remission was achieved after MTX withdrawal and treatment with anti-CD20 monoclonal antibody.

This is the first time that anti-CD20 antibodies have been used in this particular setting, without chemotherapy. Stewart et al3 recently published the case of an EBV-related non-Hodgkin's lymphoma (NHL) occurring in a patient with RA and successfully treated with Rituximab. However, this NHL occurred in the setting of a B cell chronic lymphocytic leukemia and could not be directly related to RA. Moreover, remission was achieved with both polychemotherapy and Rituximab. The LPD in our case was revealed by an autoimmune neutropenia. Lymphoproliferation was particular with an expansion of immunoblasts of B cell phenotype in the LN (Figures 1 and 2) and circulating plasma cells that initially suggested a diagnosis of plasma cell leukemia. All techniques used for clonality assessment were negative. The relation to EBV was affirmed by EBER1, 2 and BHLF-1 mRNAs expression in the LN (Figure 3) and positivity for EBV-PCR in the blood. BHLF-1 positivity indicated replicative ongoing EBV infection. We could not detect monoclonal EBV genomic material in the LN of this patient by Southern blot. The presence of anti-EBNA antibodies at diagnosis excluded a primo-infection to EBV.

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Authors and Affiliations

  1. Service d'Hématologie, Hôpital Cochin, AP-HP, Paris, France

    C Kelaïdi, C Lecoq-Lafon, F Dreyfus & D Bouscary

  2. Service d'Anatomopathologie, Hôpital Cochin, AP-HP, Paris, France

    M Tulliez

  3. Service de Rhumatologie Paris V, Hôpital Cochin, AP-HP, Paris, France

    X-V Pham & A Kahan

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Kelaïdi, C., Tulliez, M., Lecoq-Lafon, C. et al. Long-term remission of an EBV-positive B cell lymphoproliferative disorder associated with rheumatoid arthritis under methotrexate with anti-CD20 monoclonal antibody (Rituximab) monotherapy. Leukemia 16, 2173–2174 (2002). https://doi.org/10.1038/sj.leu.2402588

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