Εκδηλώσεις και ευρήματα από το συνδετικό ιστό σε ασθενείς με λεμφοϋπερπλαστικά νοσήματα

Abstract

Introduction: Several studies have linked certain autoimmune diseases to an increased occurrence of lymphoma. Immunological and molecular characteristics in such patients have not been extensively studied and the clinical significance of autoimmune background is not clear. Patients and Methods: A group of 30 patients with autoimmunity and lymphoma (group A) was studied for autoantibodies, histological subtype, disease stage, immunophenotype of the blood with flow cytometry and monoclonality of the blood (IgH or TCR rearrangement with PCR) and was compared to 30 patients with lymphoma of the same histological subtype, stage and gender without autoimmune background (group B). Results: Of the 30 patients of group A [25 with B non-Hodgkin lymphoma (B-NHL) and 5 with T non-Hodgkin lymphoma (T-NHL)], 17 were female and 13 male with a median age of 54 years (21-72). The more common underlying autoimmune diseases were Sjögren syndrome (10/30), Psoriasis, (8/30) and Rheumatoid Arthritis (5/30), followed by Cryoglobulinemia (2/30), Discoid lupus (2/30), Sarcoidosis (1/30), Mixed Connective Tissue Disease (1/30) and Graves disease (1/30). All patients were negative for Hepatitis B or C. Median time between diagnosis of the autoimmune disorder and development of lymphoma was 7.7 years (1-18). Of the patients with B-NHL17/25 (68%) developed marginal extranodal lymphoma, 6/25 (24%) Diffuse Large B Cell Lymphoma, 1/25 lymphoma from the blastic centres and 1/25 Waldenström macroglobulinemia. Sixteen patients were of early and 9 of advanced stage. Of the patients with T-NHL, 4/5 developed primary cutaneous lymphoma and 1/5 angioimmunoblastic lymphadenopathy. Three were of early and 2 of advanced stage. Immunophenotyping of the blood was performed in 20/30 patients. Abnormal phenotype consistent with the disease was demonstrated in 1 patient with B- and in 2 patients with T-NHL. CD4+ were reduced in 13/20 patients whereas HLA-DR and/or CD38+, T-LGL and NK were elevated in 7/20, 3/20 and 4/20 patients respectively. IgH rearrangement on diagnosis was demonstrated in 5/11 B-NHL patients of early stage. Characteristics of patients with autoimmunity and B-NHL (group I) and control patients (group II) are shown in table 1. Conclusions: Sjögren syndrome results more frequently to development of lymphoma. Lymphomas developing on autoimmune background are more commonly B-NHL, extranodal from the marginal zone and of early stages. Coexistence of autoimmunity and lymphoma is characterized by immunophenotypic T lymphocytes disorders though not significantly different from those observed in patients with lymphoma alone. Further studies are required to shed light on the nature of this association.