Background. Non-MALT marginal zone lymphomas (MZL) include splenic (SMZL) and nodal (NMZL) types which are rare indolent non-Hodgkin lymphomas; the incidence of autoimmune complications is reported between 10 and 15%. Aim. We performed a retrospective analysis in our patients with non-MALT MZL to evaluate the incidence of autoimmune phenomena, their relationship with clinical and biological characteristics and their impact on survival. Patients and Methods. We retrospectively evaluated 82 patients with non-MALT MZL, diagnosed between 1988 and 2007 at our Institution. Seventy-four were affected by SMZL and 8 by NMZL. In 45/74 patients with SMZL the diagnosis was made by bone marrow biopsy and in 29 by spleen and bone marrow his- tologies. The patients with NMZL were diagnosed by bone marrow biopsy in 3 cases and by lymph-node and bone marrow biopsies in the remaining ones. Fifty-two patients were males and 30 females (ratio 1,73) with a median age at diagnosis of 64 years (range 34-86). In 82% of SMZL patients splenomegaly was present, while all the NMZL patients had peripheral lymphadenopathy. Serum M-component was detectable in 29/82 (35,3%) patients. Anti-HCV seropositivity (RIBA) was found in 14/62 (22,6%) tested patients. Results. In 20/82 patients (24,4%) clinically relevant autoimmune complications occurred; we found 8 cases of autoimmune haemolytic anaemia (AIHA), 2 of autoimmune thrombocy- topenia, 2 of Evans’ syndrome, 3 of anti-MAG-associated neuropathy, and one case of rheumatoid arthritis, Sjogren’s syndrome and symptomatic crioglobulinemia respectively. Moreover one patient presented both AIHA and antiphospholipid syndrome (APS) and another autoimmune thrombocytopenia and APS. We also found 7 cases of clinically asymptomatic crioglobulinemia and 8 patients with seropositivity for Rheumatoid factor or ANA. Incidence of autoimmune complications was higher in females (p<0.05) while we found no statistically significant correlation between autoimmune disorders and: age, presence of splenomegaly, LDH or β2-microglobulin levels, anti-HCV seropositivity and leukemic presentation. In 14/20 patients autoimmune complications occurred at presentation or within 3 months from diagnosis; in 3 patients autoimmune phenomena preceded the diagnosis of lymphoma of 60, 18 and 3 months respectively. Treatment of symptomatic autoimmune complications consisted of: steroids (13 patients) associated with alkylating agents (7) or splenectomy (5), Rituximab + polichemotherapy (5), other drugs (2). Median overall survival of all patients was 108 months; the occurrence of autoimmune complications did not significantly influence survival. Conclusions. In our series of non-MALT marginal zone lym- phomas the incidence of autoimmune complications was higher than previously reported. In the majority of cases they occurred at diagnosis or even before it. Survival was not influenced by the occurrence of autoimmune phenomena.
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