Abstract
Clinical features of 40 lymphoproliferative neoplasm patients in the setting of systemic autoimmune diseases managed in the Clinic of Hematology during 1994–2006 were analyzed retrospectively. The classification of systemic autoimmune disease patients was as follows: 15 systemic lupus erythematosus—SLE, 11 rheumatoid arthritis—RA, 12 Sjögren’s syndrome—SS, 1 scleroderma, and 1 dermatomyositis. Patients comprised 31 women and 9 men of mean age 55 years (range 33–76). Systemic autoimmune diseases preceeded the development of lymphoproliferative neoplasms in 37/40 (92.5%) patients. Mean latency period between the onset of systemic autoimmune diseases and lymphoproliferative neoplasms occurrence was significantly longer in RA (113 months) than in SLE (75 months) and SS patients (65 months)—P < 0.05. The most frequent lymphoproliferative neoplasms were non-Hodgkin’s lymphoma—NHL (35/40; 88%), diffuse large B-cell lymphoma (DBCL)—12 (34%), follicular lymphoma (FC)—7 (20%), small lymphocytic (SL), and marginal zone lymphoma (MZL)—5 (14%) each. The primary site of NHL was extranodal in 18/35 (51.5%) cases. Advanced disease on diagnosis (III + IV clinical stages), constitutional symptoms, and bulky disease were diagnosed in 27/35 (77%), 26/35 (74%), and 3/35 (8.5%) patients, respectively. The overall survival (OS) was as follows (months): DBCL-12, FC-63, SLL-60, and MZL-48. There was no association between the lymphoproliferative neoplasm histological subtype and the systemic autoimmune diseases type or antirheumatic treatment P > 0.05. Our findings are in line with earlier reports showing a high proportion of patients with advanced disease, constitutional symptoms, extranodal manifestations, high grade histology, and low OS in the systemic autoimmune diseases setting.
Similar content being viewed by others
References
Zintzaras E, Voulgarelis M, Moutsopoulos HM. The risk of lymphoma development in autoimmune diseases. Arch Intern Med. 2005;165:2337–44.
Bernatsky S, et al. Non-Hodgkin lymphoma in systemic lupus erythematosus. Ann Rheum Dis. 2005;64:1507–9.
Kaiser R. Incidence of lymphoma in patients with rheumatoid arthritis: a systematic review of the literature. Clin Lymphoma Myeloma. 2008;8:87–93.
Smedby KE, et al. Autoimmune and chronic inflammatory disorders and risk of non-Hodgkin lymphoma subtype. J Nat Cancer Inst. 2006;98:51–60.
Smedby KE. et.al. Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. Blood. 2008;111:4029–38.
Hansen A, Lipsky PE, Dorner T. B-cell lymphoprolifeartion in chronic inflamatory rheumatic diseases. Nat Clin Pract Rheumatol. 2007;3:561–9.
Smedby KE, Baecklund E, Askling J. Malignant lymphomas in autoimmunity and inflammation: a review of risks, risk factors and lymphoma characteristics. Cancer Epidemiol Biomarkers Prev. 2006;15:2069–77.
Wolfe F, Michaud K. Lymphoma in rheumatoid arthritis: the effect of methotrexate and anti-tumor necrosis factor therapy in 18, 572 patients. Arthritis Rheum. 2004;50:1740–51.
Jaffe ES, Harris NL, Stein H, Vardiman JW. Pathology and genetics of tumours of hematopoietic and lymphoid tissue. World Health Organisation Classification of Tumours Lyon: IARC Press; 2001.
Kristinsson SY, et al. Genetic and immune-related factors in the pathogenesis of lymphoproliferative and plasma cell malignancies. Hematologica. 2009;94:1581–9.
Arnett FC, et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum. 1988;31:315–24.
Tan EM, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25:1271–7.
Vitali C, et al. Preliminary classification criteria for Sjögren’s syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum. 1993;36:340–7.
Masi D, et al. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum. 1980;23:581–90.
Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292:344–7.
Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975;292:403–7.
Moormeier JA, Williams SF, Golomb HM. The staging of non-Hodgkin lymphomas. Semin Oncol. 1990;17:43–50.
Lister T, et al. Report of the committee convened to discuss the evaluation and staging of patients with Hodgkin’s disease: Cotsworld meeting. J Clin Oncol. 1989;7:1630–6.
Durie BG, Salmon SE. A clinical staging system for multiple myeloma. Correlation of measured myeloma cell mass with presenting clinical features, response to treatment and survival. Cancer. 1975;36:842–54.
Shipp M, et al. A predictive model for aggressive non-Hodgkin’s lymphoma. The International Non-Hodgkin’s Lymphoma Prognostic Factors Project. N Engl J Med. 1993;329:987–94.
Solal-Ce′ligny P, et al. Follicular lymphoma international prognostic index. Blood. 2004;104:1258–65.
Smedby KE, et al. Autoimmune disorders and risk of non Hodgkin’s lymphoma subtypes: a pooled analysis within the Interlymph Consortium. Blood. 2008;111:4029–38.
Landgren O, Pfeiffer RM, Kristinsson SY, Björkholm M. Survival patterns in patients with Hodgkin’s lymphoma with a pre-existing hospital discharge diagnosis of autoimmune disease. J Clin Oncol. 2010;28:5081–7.
Anderson LA, et al. Population-based study of autoimmune conditions and the risk of specific lymphoid malignancies. Int J Cancer. 2009;125:398–405.
Xu Y, Wiernik PH. Systemic lupus erythematosus and B-cell hematological malignancy. Lupus. 2001;10:841–50.
Bernatsky S, et al. Hodgkin’s lymphoma in systemic lupus erythematosus. Rheumatology. 2007;46:830–2.
Hoshida Y, et al. Lymphoproliferative disorders in autoimmune diseases in Japan: analysis of clinicopathological features and Epstein-Barr virus infection. Int J Cancer. 2003;108:443–9.
Ekstrőm K, et al. Risk of malignant lymphomas in patients with rheumatoid arthritis and in their first-degree relatives. Arthritis Rheum. 2003;48:963–70.
Lin AY, Tucker MA (1998) Epidemiology of Hodgkin’s disease and Non-Hodgkin’s lymphomas. In: Canellos GP, Lister TA, Sklar JR. The lymphomas. 1st ed. Philadelphia: W.B. Saunders Company, pp 43–61.
Mikuls TR, et al. Prospective study of survival outcomes in non-Hodgkin’s lymphoma patients with rheumatoid arthritis. J Clin Oncol. 2006;24:1597–602.
King JK, Costenbader KH. Characteristics of patients with systemic lupus erythematosus (SLE) and non-Hodgkin’s lymphoma (NHL). Clin Rheumatol. 2007;26:1491–4.
Dawson TM, Starkebaum G, Wood BL, Willkens RF, Gown AM. Epstein-Barr virus, methotrexate and lymphoma in patients with rheumatoid arthritis and primary Sjőgren’s syndrome: case series. J Rheumatol. 2001;28:47–53.
Theander E, et al. Lymphoma and other malignancies in primary Sjőgren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis. 2006;65:796–803.
Baecklund E, et al. Association of chronic inflamation, not its treatment, with increased lymphoma risk in rheumatoid arthritis. Arthritis Rheumatol. 2006;54:692–701.
Engels EA, et al. Immune-related conditions and immune-modulating medications as risk factors for non-Hodgkin’s lymphomas: a case-control study. Am J Epidemiol. 2005;162:1153–61.
Baecklund E, et al. Lymphoma subtypes in patients with rheumatoid arthritis: increased proportion of diffuse large B cell lymphoma. Arthritis Rheum. 2003;48:1543–50.
Hellgren K, et al. Rheumatoid arthritis, treatment with corticosteroids and risk of malignant lymphomas: results from a case–control study. Ann Rheum Dis. 2010;69:654–9.
Mariette X, et al. Lymphoma in rheumatoid arthritis patients treated with methotrexate: a 3-year prospective study in France. Blood. 2002;99:3909–15.
Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjőgren’s syndrome: a multicenter, retrospective, clinical study by the European Concerted Action of Sjőgren’s syndrome. Arthritis Rheum. 1999;42:1765–72.
Cibere J, Sibley J, Haga M. Rheumatoid arthritis and the risk of malignancy. Arthritis Rheum. 1997;40:1580–6.
Acknowledgements
This study was supported by the Ministry of Science and Education of Republic of Serbia No 41004.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Suvajdzic, N., Djurdjevic, P., Todorovic, M. et al. Clinical characteristics of patients with lymphoproliferative neoplasms in the setting of systemic autoimmune diseases. Med Oncol 29, 2207–2211 (2012). https://doi.org/10.1007/s12032-011-0022-x
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12032-011-0022-x