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Rheumatology Advance Access originally published online on June 8, 2004
Rheumatology 2004 43(8):1050-1053; doi:10.1093/rheumatology/keh248
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Rheumatology Vol. 43 No. 8 © British Society for Rheumatology 2004; all rights reserved

Paper

Combined therapy with rituximab plus cyclophosphamide/doxorubicin/vincristine/prednisone (CHOP) for Sjögren's syndrome-associated B-cell aggressive non-Hodgkin's lymphomas

M. Voulgarelis, S. Giannouli, D. Anagnostou and A. G. Tzioufas

Department of Pathophysiology, Medical School, National University of Athens, Greece.

Correspondence to: M. Voulgarelis, Department of Pathophysiology, Medical School, National University of Athens, M. Asias 75, Goudi, 11527 Athens, Greece. E-mail: mvoulgar{at}med.uoa.gr

Objective. To determine the safety and therapeutic response of a regimen consisting of cyclophosphamide/doxorubicin/vincristine/prednisone (CHOP) plus rituximab in patients with Sjögren's syndrome (SS) and aggressive non-Hodgkin's lymphoma (NHL).

Methods. Four SS patients with aggressive marginal zone NHL were enrolled in this trial. All patients were classified according to the newly proposed revised European–American classification of lymphoid neoplasms. Three out of four patients also had mixed cryoglobulinaemia (MC) of type II. They were treated every 3 weeks for eight cycles of CHOP. Patients also received rituximab, at a dose of 375 mg per square metre, on day 1 of each of the eight cycles of CHOP. Four weeks after completion of the eighth course of CHOP plus rituximab and every 6 months thereafter, patients were re-evaluated for response.

Results. Complete remission of lymphoma was achieved in all four patients. The lymphoma patients remained in remission for a period of 23, 15, 12 and 10 months respectively, while certain signs and symptoms of MC type II (purpura, peripheral neuropathy and arthralgias) significantly improved with treatment. In addition, the titres of circulating cryoglobulins and RF decreased, while C4 levels returned to normal.

Conclusion. CHOP plus rituximab was well tolerated and proved effective in SS patients with aggressive NHL. Our observations may warrant a larger controlled trial to assess the effectiveness of this regimen in such patients.

KEY WORDS: Sjögren's syndrome, Lymphoma, Rituximab, Cryoglobulinaemia


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