scholarly journals Thymic Mucosa-Associated Lymphoid Tissue B-Cell Lymphoma in a Patient with Sjögren's Syndrome

2021 ◽  
Author(s):  
Ke Lan ◽  
Fan Yang ◽  
Jun Wang ◽  
Jian Zhou
Keyword(s):  
Sjögren’S Syndrome ◽  
Malt Lymphoma ◽  
Lymphoid Tissue ◽  
Mediastinal Tumor ◽  
Sjögren's Syndrome ◽  
B Cell Lymphoma ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Severe Thrombocytopenia ◽  
Sjögren‘S Syndrome

Abstract Background: Mucosa-associated lymphoid tissue (MALT) lymphoma rarely involves the thymus gland. About 5% of patients with Sjögren’s syndrome eventually develop lymphomas.Case presentation: A 52-year-old woman with Sjögren's syndrome and immunologic thrombocytopenic purpura was found to have a mediastinal tumor. Preoperative examination revealed the patient suffered from a severe thrombocytopenia and even had a rare blood group: O D+ (D+c+c+E+E+). Resection of the mediastinal tumor via video-assisted thoracic surgery (VATS) following thoroughly preparation including thrombocyte transfusion. Histopathologic examination and immunohistochemistry of the thymus tumor were both consistent with mucosa-associated lymphoid tissue (MALT) lymphoma. Conclusion:This rare case suggests that thymic MALT lymphoma can develop with an autoimmune disease such as Sjögren's syndrome, surgical resection of thymic tumor should be performed after careful preoperative preparation.

scholarly journals Breast MALT lymphoma and AL amyloidosis complicating Sjögren’s syndrome

2019 ◽  
Vol 12 (4) ◽  
pp. e227581 ◽  
Author(s):  
Nabil Belfeki ◽  
Salima Bellefquih ◽  
Anne Bourgarit
Keyword(s):  
Sjögren’S Syndrome ◽  
Malt Lymphoma ◽  
Sjögren's Syndrome ◽  
B Cell Lymphoma ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Al Amyloidosis ◽  
Careful Clinical Examination ◽  
Amyloid Light Chain ◽  
Sjögren‘S Syndrome

Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) lymphomas of the breast with mammary amyloidosis are exceedingly rare entities. This report describes the case of women with long-standing Sjögren’s syndrome presenting with breast MALT lymphoma and amyloïd light-chain (AL) amyloidosis. Breast microcalcification needle biopsy made the positive diagnosis. This unusual finding should be kept in mind. It emphasises the need for careful clinical examination of nodes and extranodal organs supposedly affected in patients with autoimmune disease.

Nodal marginal zone B-cell lymphoma associated with Sjögren's syndrome: A report of three cases

2007 ◽  
Vol 48 (6) ◽  
pp. 1222-1224 ◽  
Author(s):  
Masaru Kojima ◽  
Norihumi Tsukamoto ◽  
Yuri Miyazawa ◽  
Misa Iijima ◽  
Kazuhiko Shimizu ◽  
...  
Keyword(s):  
B Cell ◽  
Sjögren’S Syndrome ◽  
Marginal Zone ◽  
Sjögren's Syndrome ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome—clinical features

2013 ◽  
Author(s):  
Simon Bowman ◽  
John Hamburger ◽  
Elizabeth Price ◽  
Saaeha Rauz
Keyword(s):  
Salivary Gland ◽  
B Cell ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Negative Impact ◽  
B Cell Lymphoma ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Increased Risk ◽  
Sjögren‘S Syndrome

Sjögren's syndrome is a chronic, immune-mediated, condition of unknown aetiology characterized by focal lymphocytic infiltration of exocrine glands associated with dry mouth and eyes. It occurs in its own right (primary Sjögren's syndrome, pSS), or as a late feature of other rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus or scleroderma (secondary Sjögren's syndrome). There is a strong female bias. pSS typically affects women in their middle years with an estimated prevalence of 0.1–0.6%. 75% of patients have anti-Ro and/or anti-La antibodies, often with raised immunoglobulin levels (hypergammaglobulinaemia). In patients without these antibodies the diagnosis can be confirmed by salivary gland biopsy. Treatment is generally symptomatic using artificial tears, saliva replacements/stimulants and good dental hygiene. Three-quarters of patients with pSS report significant fatigue with a negative impact on quality of life. This can be the most disabling symptom. Approximately 20% of patients develop systemic features including persistent salivary gland swelling, cutaneous vasculitis, peripheral neuropathy, interstitial lung disease, autoimmune cytopenias or renal tubular acidosis. Hydroxychloroquine and corticosteroids are the most widely used therapies for systemic features. There is a 44fold increased risk of mucosa-associated lymphoid tissue (MALT) B-cell lymphoma in pSS, typically affecting the salivary glands. On account of abnormalities in the B-cell system in pSS there is current interest in the use of anti-B-cell directed monoclonal antibodies to treat pSS and a number of clinical trials are in progress. This approach is already successfully in use for treating MALT lymphoma in pSS.

Treatment of Mucosa-associated Lymphoid Tissue Lymphoma in Sjögren’s Syndrome: A Retrospective Clinical Study

2011 ◽  
Vol 38 (10) ◽  
pp. 2198-2208 ◽  
Author(s):  
RODNEY P.E. POLLARD ◽  
JUSTIN PIJPE ◽  
HENDRIKA BOOTSMA ◽  
FRED K.L. SPIJKERVET ◽  
PHILIP M. KLUIN ◽  
...  
Keyword(s):  
Parotid Gland ◽  
Disease Activity ◽  
Malt Lymphoma ◽  
Lymphoid Tissue ◽  
Watchful Waiting ◽  
Sjögren's Syndrome ◽  
Clinical Course ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

Objective.To retrospectively analyze the clinical course of patients with mucosa-associated lymphoid tissue (MALT)-type lymphoma of the parotid gland and associated Sjögren’s syndrome (SS).Methods.All consecutive patients with SS and MALT lymphoma (MALT-SS) diagnosed in the University Medical Center Groningen between January 1997 and January 2009 were analyzed. Clinical course and treatment outcome of SS and MALT lymphoma were evaluated.Results.From a total of 329 patients with SS, 35 MALT-SS patients were identified, with a median followup of 76 months (range 16–153 mo). MALT lymphoma was localized in the parotid gland in all cases. Treatment consisted of “watchful waiting” (n = 10), surgery (n = 3), radiotherapy (n = 1), surgery combined with radiotherapy (n = 2), rituximab only (n = 13), or rituximab combined with chemotherapy (n = 6). Complete response was observed in 14 patients, partial response in 1 patient, and stable disease in 20 patients. In 6 of 7 patients with initially high SS disease activity (M-protein, cryoglobulins, IgM rheumatoid factor > 100 KIU/l, severe extraglandular manifestations), MALT lymphoma progressed and/or SS disease activity increased after a median followup of 39 months (range 4–98 mo), necessitating retreatment. Only 1 patient with MALT who had low SS disease activity showed progression of lymphoma when left untreated.Conclusion.An initially high SS disease activity likely constitutes an adverse prognostic factor for progression of lymphoma and/or SS. Such patients may require treatment for both MALT lymphoma and SS. In SS patients with localized asymptomatic MALT lymphoma and low SS disease activity, a “watchful waiting” strategy seems justified.

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