Riccioni R, Caracciolo F, Galimberti S, Cecconi N, Petrini M
Haematology Division, Department of Oncology, Transplant and Advanced Technologies, University of Pisa, Italy.
Hematol Oncol. 2003 Dec;21(4):163-8. doi: 10.1002/hon.717.
Splenic Marginal Zone Lymphoma (SMZL) is a rare clinicopathological entity among marginal zone lymphomas. SMZL is an indolent lymphoma usually treated by splenectomy. A subset of patients is characterized by a more aggressive clinical course and poor prognosis. Treatment of these cases and second-line therapy for relapsed patients have not been yet identified. We report 10 cases treated with cladribrine (5 mg/m(2)/week) for six courses. Six patients (60%) achieved partial response, two patients (20%) achieved a complete response and the two remaining patients did not respond and died as a result of progression of the disease. The treatment was well tolerated. A total of 60% of the patients had an overall survival rate of 48 months and 24 months progression-free-survival was achieved by 37% with a median time of progression-free-survival of 17 months. Interestingly, in addition to a relevant percentage of hematological remission, some patients also experienced a molecular remission. We conclude that this treatment is safe and well tolerated and is able to induce a substantial number of responses. Our results suggest that this schedule is well tolerated and could be an useful alternative to splenectomy.
脾边缘区淋巴瘤（SMZL）是边缘区淋巴瘤中一种罕见的临床病理实体。SMZL是一种惰性淋巴瘤，通常通过脾切除术进行治疗。一部分患者具有更具侵袭性的临床病程和较差的预后。这些病例的治疗方法以及复发患者的二线治疗方案尚未确定。我们报告了10例接受克拉屈滨（5 mg/m²/周）治疗六个疗程的患者。6例患者（60%）达到部分缓解，2例患者（20%）达到完全缓解，其余2例患者无反应并因疾病进展而死亡。该治疗耐受性良好。共有60%的患者总生存率为48个月，37%的患者无进展生存期达24个月，无进展生存期的中位时间为17个月。有趣的是，除了相当比例的血液学缓解外，一些患者还出现了分子缓解。我们得出结论，这种治疗安全且耐受性良好，能够诱导大量缓解。我们的结果表明，该方案耐受性良好，可能是脾切除术的一种有用替代方法。
