Combination of Rituximab, Cyclophosphamide, and Vincristine Induces Complete Hematologic Remission of Splenic Marginal Zone Lymphoma

Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria

“…Under one-third of patients have a monoclonal paraprotein usually less than 20 g l À1 . 3 In a small number of patients, particularly those originating from Southern European countries, there is evidence of hepatitis C virus (HCV) infection; 4,5 it has been suggested that this virus may play a pathogenic role in disease development. Autoimmune phenomena are present in 20% of patients.…”

“…3,10,12 (b) purine analogs, in particular fludarabine (F) in combination or not with rituximab and rituximab alone have demonstrated in different studies a greater efficacy than alkylating agents in terms of quality of response and longer progression-free survival. 4,[68][69][70][71][72][73][74][75][76] Thus, although the total number of patients treated with purine analogs and/or rituximab is relatively small, these drugs seem to be very effective and warrant consideration as first-line therapy in patients who are not fit for surgery or have progressed following splenectomy.…”

Clinical and biological diversity of splenic marginal zone lymphoma

“…It appears that fludarabine has a greater activity than the other purine analogs. Excellent responses to single-agent rituximab or in combination with fludarabine or fludarabine and cyclophosphamide or cladribine have been documented [19,20,[39][40][41]. It is unknown whether chemotherapy plus rituximab is a better option than single-agent rituximab.…”

Nuclear factor- B dysregulation in splenic marginal zone lymphoma: new therapeutic opportunities

“…Therapeutic options for SMZL include splenectomy, 7,8 chemotherapy, 9,10 rituximab alone 11,12 or rituximab + chemotherapy. [11][12][13] In addition, antiviral treatment should be considered in patients with SMZL and concurrent HCV-related chronic hepatitis who do not immediately need conventional chemo/immuno-therapy against the lymphoma clone. [14][15][16] NF-kB signaling is transiently engaged when normal B lymphocytes respond to antigens, promoting cell survival and differentiation.…”

“…Yan et al 13 identified inactivating mutations of the TNFAIP3 (13%), a negative regulator of the canonical NF-kB signaling, as well as activating lesions of MYD88 (13%) and CARD11 (9%). Among their many functions, these act as positive regulators of NF-kB in signaling from B-cell and Toll-like receptors.…”