Abstract

A 66-year-old woman was diagnosed with lymphoplasmacytic lymphoma (LPL) in 2007, and her clinical stage was IIIA. She also had Sjogren syndrome and rheumatoid arthritis before. Eight courses of CPA, THP, VCR, and PSL therapy was applied. After 10 months of complete remission (CR), the relapse was revealed by CT in 2008. Fludarabine alone therapy was then introduced as a second-line therapy. Because the progression was proved after 4 courses of the therapy, re-biopsy of right inguinal lymph node (LN) showed the same diagnosis as LPL except for the CD20 positivity. The third-line chemotherapy of rituximab, IFO, MIT, VDS, and PSL was applied and completed 8 courses. After a short period of partial remission, systemic LNs enlarged gradually, and then the watch and wait strategy was chosen. In 2012 she reported fever, general fatigue, and night sweats. The examinations showed low platelet count, elevation of LDH and generalized lymphoadenopathy, especially abdominal paraaortic LNs showing higher metabolic activities in PET-CT. Right inguinal LN biopsy revealed many large B cells and plasmablastoid cells, but still the diagnosis did belong to LPL. However, we believed the clinical transformation of LPL to aggressive B cell lymphoma, possibly to diffuse large B cell lymphoma (DLBCL) because of more aggressive clinical features. Bendamustine plus rituximab was applied as fourth line. After completion of 6 courses, complete metabolic remission was confirmed by PET-CT in 2013, and the disease still keeps CR state until now. Bendamustine is now one of an active agent to relapsed or refractory indolent B cell lymphoma in Japan. Based on the knowledge published in western countries, a salvage treatment with bendamustine can be effective and safe for heavily pretreated patients with relapsed or refractory DLBCL. Our case also showed that bendamustine plus rituximab can be a promising option for the treatment of relapsed or refractory aggressive B cell lymphoma.

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