Surgery has been the treatment of choice in patients affected by BALT NHL; however, radical surgery is not always possible and does not avoid the risk of relapse. Systemic treatment is recommended for symptomatic patients who relapse after surgery or cannot undergo such procedure; nevertheless, there is no standard systemic chemotherapy which could be recommended for such indolent disease. Here we report clinical data of 16 patients (9 men and 7 female) with biopsy-proven low grade BALT lymphoma followed at EIO and IOSI from 1992 to 2004. Median age was 62 years (range 50–77); 8 patients presented symptomatic disease at diagnosis. Thoracotomy was necessary to obtain histological material in eight patients, while in the other 8 no-invasive procedures were performed. Thirteen patients were considered in stage IE and 3 in stage IV (bone marrow involvement in 2 patients, gastric in 1), according to Ann Arbor. Five patients received upfront aggressive surgery: 1 underwent wedge resection and 4 lobectomy. Surgery was the definitive treatment for 3 patients. With a median follow-up of 17 months (range 9–31), all these patients are still alive with no evidence of disease. Twelve patients received systemic treatment: two because of non-radical surgery and ten because of advanced stage, symptomatic disease or psychosocial reasons. The majority of them (7 patients) received alkylating containing regimen (Chlorambucil ± PDN), alone or in combination with Rituximab (Mabthera) (2 patients). Three additional patients, refusing CT, received Rituximab alone in a standard schedule. Hematological and non-hematological toxicity was in general mild: transient G3 neutropenia occurred in only one patient. No patient discontinued treatment because of treatment-related toxicity and no toxic death was recorded. All patients responded to systemic therapy with five patients achieving CR. Four of them are still alive and free of disease after 24, 65, 27, 42 months respectively. One patients still in CR died for reasons different from lymphoma after 54 months. With a median follow-up of 60 months (range 31 – 63), only four patients developed progressive disease or relapse after 67, 5, 63, 18 months respectively (median TTP=40 months). Our experience suggests that systemic treatment, when appropriate, is able to achieve an high response rate in this relative uncommon disease. Considering the clinical results and the low degree of toxicity observed, alkylating containing regimen (Chlorambucil ± PDN) alone or in combination with monoclonal antibody could be considered as first line treatment for patients with BALT lymphoma.

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