• linfoma
• marginale
• splenico

Splenic marginal zone lymphoma (SLVL/SMZL) is an indolent lymphoproliferative disease that typically affects elderly patients and that has a median survival greater than 10 years. The percentage of patients surviving 5 years from diagnosis is 65 to 75%, often even in the absence of treatment or of a complete response to therapy. To date, standard criteria inducing the clinician to treat SMZL are not yet defined. There is no clear advantage to the early therapy, that is considered indicated when patients developed significant signs and/or symptoms. On this light “a watch and wait” policy is frequently employed and it is also reasonable for asymptomatic patients who have moderate cytopenias and no-bulky splenomegaly.
No specific prognostic factors have been established for SMZL.
Intergruppo Italiano Linfomi (IIL) defined a prognostic score system that include LDH, albumin and haemoglobin levels identifying 3 different risk groups: low, intermediate and high.
When splenomegaly is symptomatic splenectomy is the treatment of choice with consequent improvement of performance status and correction of cytopenias.
However, patients unfit for surgery because of comorbidity or high tumor burden need of a systemic therapy. Immunochemotherapy represents the best approach.
Purine analogues are frequently used in combination with rituximab in these patients with great results. The use of such schemes , however, is not recommended in patients with a history of malignancy or recurrent infectious episodes .
In this paper the association between oral cyclophosphamide and Rituximab has been evaluated as possible alternative therapeutic choice in SMZL patients not eligible for surgery or more toxic regimens.