Br J Haematol 1991 Jun;78(2):206-9 Related Articles, Links
Splenic lymphoma with villous lymphocytes: natural history and response to therapy in 50 cases.
Mulligan SP, Matutes E, Dearden C, Catovsky D.
Academic Department of Haematology and Cytogenetics, Royal Marsden Hospital, London.
We studied the natural history and response to treatment in 50 patients with splenic lymphoma with villous lymphocytes followed for a minimum of 6 months and up to 15 years (median 3.7 years). The disease occurs in the elderly (median 68 years) and affects males more than females (M/F ratio 1.77). The median survival for the group was not reached but 82% were surviving at 3 years and 78% at 5 years. Twelve patients (24%) died, one-third of deaths were disease related (four patients) and one-half were due to cardiovascular disease or another malignancy (six patients). The outcome was worse for males (31% died) than females (11% died). Fourteen patients were not treated and 10 remain alive between 1 and 6 years from diagnosis. The remaining patients were treated by chemotherapy, splenic irradiation or splenectomy. The response to chemotherapy was poor and only eight of 22 (36%) patients treated achieved a good response. Splenic irradiation was employed in seven patients and three benefited from it. Splenectomy seems to be the treatment of choice, with significant improvement seen in 19 of 20 patients with one post-operative death. This response, lasting from 6 months to 7 years (median 4 years), was seen irrespective of whether splenectomy was the initial treatment or used later in management.
JCO 20(18):3872, 2002
Prospective study to evaluate the activity of the nucleoside analog cladribine 2-CdA in extranodal MALT.
PATIENTS AND METHODS: Patients with histologically verified MALT-type lymphoma were enrolled. 2-CdA was administered at a dose of 0.12 mg/kg body weight on 5 consecutive days, as a 2-hour infusion. Cycles were repeated every 4 weeks for a maximum of six cycles.
RESULTS: Nineteen patients with gastric and seven patients with extragastric MALT lymphoma were enrolled. All patients were chemotherapy-naive, and two had been locally irradiated before systemic relapse of the lymphoma. A total of 102 cycles was administered to our patients (median number of cycles per patient, four). All 25 assessable patients responded to treatment: 21 patients (84%) achieved complete remission (CR) and four patients achieved partial remission. All patients (100%) with gastric presentation, but only three patients (43%) with extragastric presentation, achieved CR. Toxicities were moderate and mainly hematologic and required dose reduction and/or premature discontinuation of therapy in only three cases. Two patients died from vascular events, one shortly after the first cycle because of myocardial infarction and the other from stroke 3 months after the second course. Three patients relapsed after 13, 18, and 22 months and one patient showed progressive disease after 15 months. At present, 24 patients are alive at a median follow-up time of 32 months.
CONCLUSION: Our data demonstrate that 2-CdA is highly effective in inducing CR in 84% of patients with MALT-type lymphoma