Introduction: Splenic (SMZL) and nodal marginal zone lymphomas (NMZL) are rare indolent disorders with a reported incidence of autoimmune complications (AIC) of 10 to 15%.We retrospectively evaluated our series of SMZL and NMZL to assess the frequency and characteristics of AIC, their relationship with clinical and biological parameters and their impact on the disease course. Patients and Methods: We analyzed 143 patients, 86 males, 57 females, median age 63, diagnosed at our Institutions. SMZL were 117, NMZL 26. Diagnosis was based on histological and immunohistological analysis of spleen (45 cases) and/or bone marrow in SMZL, of lymphnodes in NMZL. Anti-HCV seropositivity (RIBA) was detected in 26/122 (21.3%) tested patients. Results: Clinically relevant AIC occurred in 34/143 (23.8%) patients: autoimmune haemolytic anaemia (AIHA) in 12, autoimmune thrombocytopenia in 6, Evans’ S. in 2, autoimmune pancytopenia in 2, anti-MAG neuropathy in 3, Sjoegren’s S. in 3, rheumatoid arthritis in 2, symptomatic crioglobulinemia in 2, Sweet S. and glomerulonephritis in 1 respectively. In 2 cases with AIHA antiphospholipid syndrome coexisted. Of the remaining 109 patients, 88 were laboratory screened for autoimmunity; 26 of them (29.5%) were positive (Rheumatoid Factor or ANA 11, asymptomatic crioglobulinemia 11, LAC 4). The incidence of AIC was significantly higher in females and in patients aged <60 years. No significant correlations were found between AIC and: spleen size, LDH or beta2-microglobulin levels, anti-HCV seropositivity and leukemic phase. In 19/34 patients AIC occurred at presentation, in 7 they preceded the diagnosis of lymphoma of 3 to 94 months. Symptomatic AIC were treated with: steroids ± alkylating agents (16), splenectomy (5), Rituximab ± chemotherapy (4), alkylating agents (2), other drugs (2). Median survival of all patients was 113 months; it was not significantly influenced by the occurrence of AIC. Conclusions: Clinical and laboratory autoimmune phenomena were frequent in our series of SMZL and NMZL, overall affecting more than 40% of patients and mostly occurring at diagnosis or even before, but apparently not influencing survival.
Autoimmune complications are frequent in splenic and nodal marginal zone lymphomas
BONIFACIO, Massimiliano;AMBROSETTI, Achille;CHILOSI, Marco;PERBELLINI, Omar;ZAMO', Alberto;PIZZOLO, Giovanni
2008-01-01
Abstract
Introduction: Splenic (SMZL) and nodal marginal zone lymphomas (NMZL) are rare indolent disorders with a reported incidence of autoimmune complications (AIC) of 10 to 15%.We retrospectively evaluated our series of SMZL and NMZL to assess the frequency and characteristics of AIC, their relationship with clinical and biological parameters and their impact on the disease course. Patients and Methods: We analyzed 143 patients, 86 males, 57 females, median age 63, diagnosed at our Institutions. SMZL were 117, NMZL 26. Diagnosis was based on histological and immunohistological analysis of spleen (45 cases) and/or bone marrow in SMZL, of lymphnodes in NMZL. Anti-HCV seropositivity (RIBA) was detected in 26/122 (21.3%) tested patients. Results: Clinically relevant AIC occurred in 34/143 (23.8%) patients: autoimmune haemolytic anaemia (AIHA) in 12, autoimmune thrombocytopenia in 6, Evans’ S. in 2, autoimmune pancytopenia in 2, anti-MAG neuropathy in 3, Sjoegren’s S. in 3, rheumatoid arthritis in 2, symptomatic crioglobulinemia in 2, Sweet S. and glomerulonephritis in 1 respectively. In 2 cases with AIHA antiphospholipid syndrome coexisted. Of the remaining 109 patients, 88 were laboratory screened for autoimmunity; 26 of them (29.5%) were positive (Rheumatoid Factor or ANA 11, asymptomatic crioglobulinemia 11, LAC 4). The incidence of AIC was significantly higher in females and in patients aged <60 years. No significant correlations were found between AIC and: spleen size, LDH or beta2-microglobulin levels, anti-HCV seropositivity and leukemic phase. In 19/34 patients AIC occurred at presentation, in 7 they preceded the diagnosis of lymphoma of 3 to 94 months. Symptomatic AIC were treated with: steroids ± alkylating agents (16), splenectomy (5), Rituximab ± chemotherapy (4), alkylating agents (2), other drugs (2). Median survival of all patients was 113 months; it was not significantly influenced by the occurrence of AIC. Conclusions: Clinical and laboratory autoimmune phenomena were frequent in our series of SMZL and NMZL, overall affecting more than 40% of patients and mostly occurring at diagnosis or even before, but apparently not influencing survival.
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