Risk factors for malignant lymphoproliferation in Sjögren’s syndrome


Authors: H. Dejmková 1;  R. Bečvář 1;  J. Střítecký 2;  M. Hnátková 3;  E. Žikešová 3;  M. Trněný 3
Authors‘ workplace: Revmatologický ústav, Praha, 2Ústav patologie, Praha, 3I. interní klinika UK, Praha 1
Published in: Čes. Revmatol., 15, 2007, No. 3, p. 158-161.
Category: Case Report

Overview

Sjögren’s syndrome is a chronic autoimmune disease characterized by lymphoproliferative infiltration of exocrine glands. Infiltration leads to deterioration of gland and alteration of its function such as loss of tears and decreased salivary excretion. Course of the disease is characterized by either local autoimmune process or systemic disease affecting many internal organs. Regarding the fact that Sjögren’s syndrome is mostly associated with malignant transformation to lymphoproliferative disease, it is considered as a borderline disease between autoimmunity and malignant lymphoproliferation. The results of several studies evaluating risk factors for malignant transformation are inconsistent. The enlargement of parotid gland, lymphadenopathy, palpable purpura, cutaneous vasculitis and neuropathy belong to the most frequently referred clinical risk factors. Lymphocytopenia, decline in CD4 positive lymphocytes, hypogammaglobulinemia, monoclonal gammapathy, cryoglobulinemia and low levels of complement C3 and C4 represent the most frequently referred laboratory risk factors. Presented case report describes most of abovementioned risk factors in a patient who demonstrated transformation from primary Sjögren’s syndrome to diffuse large B-cell non-Hodgkin lymphoma with kappa positive plasmocellular differentiation.

Key words:
Sjögren’s syndrome, lymphoma, risk factors for malignant transformation


Sources

1. Vitali C, Bombardieri S, Jonnsson R, et al. European Study Group on Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Concensus Group. Ann Rheum Dis 2002; 61: 554–8.

2. McAvoy BA, et al. Sjögren’s syndrome II. Clinical associations and immunological phenomena.Q J Med 1973; 42: 513–48.

3. Moutsopoulos HM, Chused TM, Mann DL, et al. Sjögren’s syndrome (sicca syndrome): current issues. Ann Intern Med 1980; 92: 212–26.

4. Mičeková D, Rovenský J, Lukáč J, et al. Sjögrenův syndrom In Pavelka K, Rovenský J. Klinická revmatologie. Praha: Galén, 2003, 303–9.

5. Scofield RH, Bruner GR, Harley JB, et al. Autoimmune thyroid disease is associated with a diagnosis of secondary Sjögrenś syndrome in familial systemic lupus. Ann Rheum Dis 2007; 66: 410–3.

6. Scofield RH. Autoimmune thyreoid disease in systemic lupus erythematodes and Sjögren’s syndrome. Clin Exp Rheumatol 1996; 14: 321–30.

7. Saito M, Fukuda T, Shiohara T, et al. Angioimmunoblastic T-cell lymphoma: A relatively common type of T-cell lymphoma in Sjögren’s syndrome. Clin Exp Rheumatol 2005; 23: 888–90.

8. Voulgarelis M, Dafni UG, Isenberg DA, et al. Malignant lymphoma in primary Sjögren’s syndrome. A multicenter, retrospective, clinical study by the European Concerted action on Sjögren’s syndrome. Arthritis Rheum 1999; 8: 1765–72.

9. Suitcliffe N, Inanc M, Speight P, et al. Predictors of lymphoma development in primary Sjögren’s syndrome. Semin Arthritis Rheum 1998; 28: 80–7.

10. Tzioufas GA. B-cell lymphoproliferation in primary Sjögren’s syndrome. Clin Exp Rheumatol 1996;14 (Suppl)14: 65–70.

11. Valesini G, Priori R, Bavoillot D, et al. Differential risk of non-Hodgkin’s lymphoma in Italian patients with primary Sjögren’s syndrome. J Rheumatol 1997; 24: 2376–80.

12. Manganelli P, Fietta P, Quaini F. Hematologic manifestations of primary Sjögren’s syndrome. Clin Exp Rheumatol 2006; 24: 438–48.

13. Kassan SS, Thomas TL, Mounsopoulos HM, et al. Increased risk of lymphoma in sicca syndrome. Ann Intern Med 1978; 89: 888–92.

14. Whaley K, Webb J, Theander E, Henriksson G, Ljunberg O, et al. Lymphoma and other malignancies in primary Sjögren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis 2006;65: 796–803

15. Masaki Y, Sugai S. Lymphoproliferative disorders in Sjögren’s syndrome. Autoimun Rev 2004;3: 175–82.

16. Kruize AA, Hené RJ, Van Der Heide A, et al. Long-term follow-up of patiens with Sjögren’s syndrome. Arthritis Rheum 1996;39: 297–303.

17. Seror R, Sordet CH, Guillevin L, et al. Tolerance and efficacy of rituximab and changes in serum B cell biomarkers in patiens with systemic complications of primary Sjögrenś syndrome. Ann Rheum Dis 2007; 66: 351–7.

18. Mortlock AM, Lim CSE, Morgan H, et al. Renal MALToma: an unusual lymphoma in a patient with lupus. Lupus 2006; 15: 613–5.

19. Tzioufas AG, Bomba DS, Skopouli FN, et al. Mixed monoclonal cryoglobulinemia and monoclonoal rheumatoid factor cross- reactive idiotypes as predictive factors for the development of lymphoma in primary Sjögren’s syndrome. Arthritis Rhem 1996; 39: 767–72.

20. Klener P. Myeloproliferativní onemocnění in Klener P., et al. Vnitřní lékařství. Praha: Galén, 2006, 458–483.

21. Ionnidis JAP, Vassiliou VA, Moutsopoulos M. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren’s syndrome. Arthritis Rheum 2002; 46 (3): 741–7.

22. Anaya JM, McGuff HS, Banks PM, et al. Clinicopathological factors relating malignant lymphoma with Sjögren’s syndrome. Semin Arthritis Rheum 1996;25: 337–46.

23. Ramos-Casals M, Font J, Garcia-Carrasco M, et al. Primary Sjögren’s syndrome. Hematologic patterns of disease expression. Medicine 2002; 81: 281–92.

Labels
Dermatology & STDs Paediatric rheumatology Rheumatology
Login
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account