Long-term remission of Sjogren's syndrome-associated aggressive B-cell non-Hodgkin's lymphomas following administration of combined B-cell depletion therapy and CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone)

Michael Voulgarelis ¹, Stavroula Giannouli ¹, Athanasios G Tzioufas ¹ and Haralampos M Moutsopoulos ^1*

¹ Department of Pathophysiology, Medical School, National University of Athens, Greece

^* To whom correspondence should be addressed. E-mail: hmoutsop{at}med.uoa.gr.

Accepted 27 November 2005

Abstract

OBJECTIVE: Primary Sjogren's syndrome (SS) is associated with an increased frequency of NHLs mainly of low histologic grade. However, aggressive diffuse large B-cell lymphomas (DLBCL) characterized by poor treatment outcome could also be encountered in SS patients. It has recently been proven, that rituxan has significant therapeutic activity in this type of lymphoma. We conducted an open-single case trial to evaluate a) the efficacy of CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisone) in combination with rituxan in SS patients with DLBCL and b) the outcome of the patients.

METHODS: Six SS patients with DLBCL were assigned to receive eight cycles of CHOP every three weeks plus rituxan given on day 1 of each cycle. In the retrospective study, conducted by the European Concerted Action for SS, 9 cases were diagnosed as DLBCL, all of whom had been treated with CHOP alone. All these patients were used as historical controls.

RESULTS: The difference in the overall survival between the two treatment groups was significant. The group treated with rituxan/CHOP showed 100% 2 year overall survival rate, whereas historical controls showed only 37%. Extraglandular manifestations serving as predictors for lymphoma development such as palpable purpura and peripheral neuropathy disappeared. The remission of these signs was accompanied by a decrease in both circulating monoclonal cryoglobulins and rheumatoid factor activity and an increase in C4 levels. Clinically relevant toxicity was not detected.

CONCLUSIONS: The addition of rituxan to standard CHOP chemotherapy contributes to an improved treatment outcome in SS patients with aggressive DLBCL, without increasing toxicity.

Keywords: Sjogren's syndrome, diffuse large B-cell lymphoma, non-Hodgkin's lymphoma

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