Mucosa-Associated Lymphoid Tissue Lymphoma in Sjögren's Syndrome: Risks, Management, and Prognosis
Section snippets
Mucosa-associated lymphoid tissue lymphoma in Sjögren's syndrome: clinical aspects and histopathology
NHL has a 4.3% prevalence in patients who have SS. In these patients the median age at lymphoma diagnosis is 58 years, and the median time from SS diagnosis to lymphoma diagnosis is 7.5 years.3, 12 Various histologic subtypes of NHL occurring in patients who have SS have been described in the literature, including follicle center lymphoma, lymphoplasmacytoid lymphoma, and diffuse large B-cell lymphoma (DLBCL), but mucosa-associated lymphoid tissue (MALT) lymphomas are by far the most common.3, 4
Clinical and serologic risk factors of lymphoma development in Sjögren's syndrome
Despite its unequivocal association with SS, the development of lymphoma constitutes a complication rather than the rule. Only 5% of patients who have SS ultimately manifest lymphoma. Several investigations have attempted to establish predictive markers for this progression. In 1971 Anderson and Talal29 showed that a decrease in the level of serum immunoglobulins and disappearance of rheumatoid factor coincided with the time of progression to lymphoma. Kassan and colleagues3 showed that
Management of mucosa-associated lymphoid tissue lymphomas associated with Sjögren's syndrome
Large series have demonstrated that nongastric MALT lymphomas in patients who do not have SS seem to have a good outcome, with a 5-year overall survival rate from diagnosis ranging from 86% to 100%.37, 38 Although 30% of these patients presented with disseminated disease, their outcome remained unaffected by the multifocal nature of the lymphoma.28 The finding that patients presenting with extranodal MALT lymphoma affecting multiple mucosal sites have a favorable outcome with survival curves
Prognostic factors
The overall survival of patients who have MALT lymphoma is 85% to 95% at 5 years, with little difference between sites.28, 38, 51 Regardless of site, approximately 25% to 35% of patients relapse after a CR, often late and to further extranodal or nodal sites, although significantly more relapses occur in nongastric cases than in gastric cases.37, 52 In MALT lymphomas in general, however, the 10-year expected survival rate is greater than 75%.28 Several investigations have attempted to establish
Summary
Patients who have SS and who have high risk factors such as palpable purpura, low C4 levels, and MMC constitute a separate subgroup that should be monitored more closely than other patients who have SS. These patients seem to have a strong predisposition for the development of lymphoproliferation, especially low-grade extranodal MALT lymphomas. Therefore, the clinical follow-up of patients who have SS should include routine complement determination and serum immunoelectrophoresis to detect the
References (59)
- et al.
Predictors of lymphoma development of primary Sjogren's syndrome
Semin Arthritis Rheum
(1998) - et al.
Lymphomas in patients with Sjogren's syndrome are marginal zone B-cell neoplasms, arise in diverse extranodal and nodal sites and are not associated with viruses
Blood
(1997) - et al.
Primary B-cell lymphoma of salivary glands and its relationship to myoepithelial sialadenitis
Hum Pathol
(1988) - et al.
Intestinal dissemination of gastric mucosa-associated lymphoid tissue lymphoma
Blood
(1996) - et al.
Deletion analysis of the p16 tumor suppressor gene in gastrointestinal mucosa-associated lymphoid tissue lymphomas
Gastroenterology
(1997) - et al.
Mucosa-associated lymphoid tissue lymphoma is a disseminated disease in one third of 158 patients analyzed
Blood
(2000) - et al.
Clinical evolution, and morbidity and mortality of primary Sjogren's syndrome
Semin Arthritis Rheum
(2000) - et al.
Clinicopathological factors relating malignant lymphoma with Sjogren's syndrome
Semin Arthritis Rheum
(1996) - et al.
Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue
Blood
(2003) - et al.
Clinical activity of rituximab in extranodal marginal zone B-cell lymphoma of MALT type
Blood
(2003)
The gastric marginal zone B-cell lymphoma of MALT type
Blood
Gastrointestinal malignant lymphomas of the mucosa-associated lymphoid tissue: factors relevant to prognosis
Gastroenterology
Gastric B-cell mucosa-associated lymphoid tissue (MALT) lymphoma. Clinicopathological study and evaluation of the prognostic factors in 143 patients
Ann Oncol
Resistance of t(11;18) positive gastric mucosa-associated lymphoid tissue lymphoma to Helicobacter pylori eradication therapy
Lancet
Rheumatic diseases and malignancy—is there an association?
Scand J Rheumatol
Development of malignant lymphoma in the course of Sjogren's syndrome
Trans Assoc Am Physicians
Increased risk of lymphoma in sicca syndrome
Ann Intern Med
High incidence of free monoclonal lambda light chains in the sera of patients with Sjogren's syndrome
J Immunol
Cryoglobulinemia in autoimmune rheumatic diseases. Evidence of circulating monoclonal cryoglobulins in patients with primary Sjogren's syndrome
Arthritis Rheum
Differential risk of non-Hodgkin's lymphoma in Italian patients with primary Sjogren's syndrome
J Rheumatol
Evidence that the malignant lymphoma of Sjogren's syndrome is a monoclonal B-cell neoplasm
N Engl J Med
Development of malignant lymphoma in myoepithelial sialadenitis (Sjogren's syndrome)
Virchows Arch A Pathol Anat Histol
High frequency of t(14;18) translocation in salivary gland lymphomas from Sjogren's syndrome patients
J Exp Med
The risk of lymphoma development in autoimmune diseases: a meta-analysis
Arch Intern Med
Malignant lymphoma in primary Sjogren's syndrome: a multicenter, retrospective, clinical study by the European concerted action on Sjogren's syndrome
Arthritis Rheum
Lymphomas in patients with Sjogren's syndrome: review of the literature and physiopathologic hypothesis
Leuk Lymphoma
Most cases of primary salivary mucosa-associated lymphoid tissue lymphoma are associated either with Sjoegren syndrome or hepatitis C virus infection
Br J Haematol
Autoimmune and chronic inflammatory disorders and risk of non-Hodgkin lymphoma by subtype
J Natl Cancer Inst
The Non-Hodgkin's Lymphoma Classification Project. A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma
Blood
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2021, Journal of Oral and Maxillofacial SurgeryPathophysiologic role of Interleukin-33/ST2 in Sjögren's syndrome
2021, Autoimmunity ReviewsCitation Excerpt :This holds true especially in early disease where the symptoms and signs are usually mild and might explain the time delay before a diagnosis of SS. The importance of making the diagnosis of pSS is cardinal because of the high risk of developing lymphoma and serious systemic complications [11,12]. The morbidity of patients suffering from SS is debilitating, ranging from severe fatigue to organ impairment.
Marginal zone B-cell lymphoma: lessons from Western and Eastern diagnostic approaches
2020, PathologyCitation Excerpt :Interestingly, the occurrence of ocular adnexal MALT lymphoma in the setting of pre-existing IgG4-related disease has been highlighted in East Asia, emphasising the need for screening IgG4 expression in plasma cells accompanying such disorders (Fig. 5).25,28,82–91 MALT lymphoma is most common in the salivary glands, especially among patients with Sjögren syndrome, in which the risk of developing lymphoma is 5–20-fold higher than the general population.92,93 Early MALT lymphoma lesions arising in the salivary glands consist of ‘collars’ of B-cells, often with a monocytoid appearance, arranged around frequently obliterated ducts; this feature is not observed in myoepithelial sialadenitis of Sjögren syndrome, when it is not associated with lymphoma.
Sjögren Syndrome
2019, Clinical Immunology: Principles and PracticeMalignant Neoplasms of the Salivary Glands
2019, Head and Neck Pathology: A Volume in the Series: Foundations in Diagnostic PathologyExtranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)
2018, Atlas of Hematopathology: Morphology, Immunophenotype, Cytogenetics, and Molecular Approaches, Second Edition