Τίτλος:
Malignant lymphoma in primary Sjögren's syndrome: An update on the pathogenesis and treatment
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Objectives: Sjögren's syndrome (SS), a chronic autoimmune disorder, particularly compromises the function of exocrine glands. Its association with lymphoma is well documented. Our aim was to systematically review the molecular, clinical, histopathologic, and therapeutic aspects of these SS-related malignant lymphoproliferations. Methods: The literature was searched for original articles published between 1968 and 2012 focusing on the risk factors for lymphoma development in Sjögren's syndrome using MEDLINE and PubMed. The search terms we used were "Sjögren's syndrome," "lymphoma," and "risk factors." All papers identified were English-language, full-text papers. Results: A low-grade marginal-zone lymphoma related to mucosa-associated lymphoid tissue is the commonest lymphoid neoplasia in SS. The majority of SS-associated lymphomas are characterized by localized stage, indolent clinical course, and recurrence in other extranodal sites. Although the transition from a chronic inflammatory condition to malignant lymphoma is a multistep process that is yet poorly understood, there is increasing evidence that chronic antigenic stimulation by an exoantigen or autoantigens plays an essential role in the development of SS-associated lymphoproliferation. Conclusions: This review discusses the pathogenetic aspects of lymphomagenesis in SS. Recent advances in the treatment of lymphoma in SS are also stated. © 2013 Elsevier Inc.
Συγγραφείς:
Routsias, J.G.
Goules, J.D.
Charalampakis, G.
Tzima, S.
Papageorgiou, A.
Voulgarelis, M.
Περιοδικό:
Seminars in Arthritis and Rheumatism
Λέξεις-κλειδιά:
B cell activating factor; cyclophosphamide; doxorubicin; prednisone; rituximab; vincristine, article; B lymphocyte differentiation; cancer combination chemotherapy; cancer grading; cancer prognosis; cancer risk; disease association; disease free survival; event free survival; histopathology; human; immune dysregulation; immunopathogenesis; International Prognostic Index; large cell lymphoma; lymphocyte proliferation; lymphocytic infiltration; lymphoma; marginal zone lymphoma; molecular pathology; nonhuman; overall survival; priority journal; risk assessment; risk factor; Sjoegren syndrome; tumor localization, Autoimmunity; Lymphoproliferation; Non-Hodgkin lymphoma; Salivary glands; Sjögren's syndrome, B-Lymphocytes; Humans; Lymphoma; Risk Factors; Sjogren's Syndrome
DOI:
10.1016/j.semarthrit.2013.04.004
