Lack of efficacy of 2-chlorodeoxyadenoside in the treatment of splenic lymphoma with villous lymphocytes

Splenic lymphoma with villous lymphocytes (SLVL) is a B-cell chronic lympho proliferative disorder. Splenectomy and/or chlorambucil (CLB) are usually r egarded as the most effective treatment in SLVL patients. However, a few pa tients relapse and the second line therapy remains questionable. Although 2 -Cda has been evaluated in patients with chronic lymphoid leukemia (CLL) an d hairy cell leukemia (HCL), it has been reported as the treatment of SLVL in only one case report. Therefore, we have evaluated its efficacy and toxi city in 7 SLVL patients. The median duration between diagnosis and treatmen t was 18 months (range, 1 to 59), The patients received 2-CdA (0.1 mg/kg/d) by venous infusion for 7 days with a median number of 1 cycle (range, 1 to 2) either as a first line therapy tone patient) or after a failure of othe r therapies (splenectomy, chemotherapy). Two patients achieved a complete response. The first one maintained his CR during a follow-up of 9 months and then relapsed; the second patient remain ed in CR after a follow-up of 20 months. Four patients achieved a partial r esponse and relapsed after a median follow-up of 3.5 months (range, 1 to 4) , One patient had no response. The treatment was not well tolerated with ma ny infectious events. In the limits of our study, 2-Cda does not appear to be efficient therapy for SLVL and is not well tolerated for patients in rel apse after splenectomy or resistant to CLB.