Thymic and pulmonary mucosa-associated lymphoid tissue lymphomas in a patient with Sjögren's syndrome and literature review

Abstract: Mucosa‐associated lymphoid tissue lymphoma (MALToma) has been reported in several organs. Among MALTomas, thymic and pulmonary MALTomas are rare. The present report describes a patient with Sjögren’s syndrome who presented thymic and pulmonary MALTomas. Although the exact pathogenetic relationship between these two tumours is uncertain, it is likely that the underlying immune dysregulation related to Sjögren’s syndrome contributed to the occurrence and the unusual manifestation of MALTomas in this patient.     

Mucosa-associated lymphoid tissue lymphoma with Sj?gren's syndrome]

A 68-year-old woman presented with Sj?gren's syndrome. Chest X-ray films disclosed consolidated shadows in the right S2 and an infiltration shadow in the right S8 with small nodules. Pathological examination of transbronchial lung biopsy (TBLB) specimens revealed lymphocytic infiltrations that stained positive with UCHL-1 and L 26 in immunohistochemical studies. Lung tissue specimens obtained by video-assisted thoracic surgery showed lympho-epithelial lesions with dense lymphocytic infiltration. Southern blot hybridization and polymerase chain reaction (PCR) assays demonstrated monoclonality and immunoglobulin heavy chain gene rearrangement. These findings yielded a diagnosis of mucosa-associated lymphoid tissue (MALT) lymphoma. The detection of rear-ranged genes encoding for immunoglobulin heavy chains is useful for the diagnosis of primary pulmonary lymphoproliferative disorders, especially malignant lymphomas.     

Bronchus-associated lymphoid tissue lymphoma in a patient with primary Sjögren’s syndrome

Despite its infrequent occurrence, the possible development of lymphoma or lymphoid lung disease in patients with Sjögren’s syndrome should always be borne in mind. We describe a case of bronchus-associated lymphoid tissue (BALT) lymphoma in a patient with Sjögren’s syndrome that clearly exemplifies the wide-ranging problems of diagnosing, treating and following such patients. This difficulty is due to the fact that the clinical and radiological findings suggest interstitial lung disease, and histological assays are required for a correct diagnosis. A precise knowledge of the various histological and radiological patterns of lung involvement can aid patient management.     

Primary hepatic lymphoma in a patient with Sjögren's syndrome

Received: October 24, 2000 / Accepted: January 26, 2001     

Membraneous glomerulonephritis and non-Hodgkin's lymphoma in a patient with primary Sjögren's syndrome

The most common renal manifestation of Sj?gren's syndrome is tubulointerstitial nephritis, and glomerular disease is rare (3). A 62-year-old woman with primary Sj?gren's syndrome developed nephrotic syndrome. Kidney biopsy was consistent with membraneous glomerulonephritis. Steroid pulse therapy was not effective. Three months later she was diagnosed with non-Hodgkin's lymphoma of the tongue, and she was given CHOP therapy and radiation. Both the lymphoma and membraneous glomerulonephritis were resolved.     

Mucosa-associated lymphoid tissue lymphoma in Sjögren's syndrome: risks, management, and prognosis

Sj?gren's syndrome is a chronic inflammatory disease primarily affecting the exocrine glands. Its association with lymphoma is well documented, with salivary extranodal marginal zone lymphomas of the mucosa-associated lymphoid tissue type being the most common and constituting a major disease complication. These neoplasms are antigen-stimulated B-cell lymphomas characterized by localized stage, indolent clinical course, and recurrence in other extranodal sites. This article presents a review of the literature and discusses the clinical, histopathologic, therapeutic, and prognostic aspects of these tumors in Sj?gren's syndrome. In addition, it highlights the predictor markers of lymphoma development.     

Pulmonary MALT lymphoma with amyloid production in a patient with primary Sjögren's syndrome

A 53-year-old woman was admitted to our hospital complaining of cough, low grade fever, chest pain and sicca symptoms. A chest radiograph showed an abnormal shadow and chest computed tomography revealed a tumor in left S6. She was diagnosed as Sj?gren's syndrome by sialography and histological findings of labial biopsy. The surgically resected tumor specimen showed proliferation of lymphoid cells with lymphoepithelial lesions, which were positive for CD20 and kappa light chain. Kappa light chain-positive amyloid was found within the tumor. The tumor showed rearranged kappa light chain genes. The diagnosis was pulmonary mucosa associated lymphoid tissue lymphoma with amyloid production.     

Cutaneous T cell lymphoma in a patient with primary biliary cirrhosis and secondary Sjögren's syndrome

We describe a patient with primary biliary cirrhosis (PBC) and secondary Sjogren's syndrome (SS) with pulmonary involvement who developed a cutaneous T cell lymphoma. The clinical course of secondary SS in PBC is thought to be less complicated than in progressive systemic scleroderma and SS. In contrast to secondary SS, the risk for developing non-Hodgkin's lymphoma is highly increased in patients with primary SS. Moreover, these lymphomas are usually of B cell origin. There are few reports of T cell lymphoma in primary SS. The occurrence of a T cell lymphoma in a patient with PBC and secondary SS indicates the necessity to investigate lymphoma in patients with secondary SS.     

Cutaneous mastocytosis in a patient with primary Sjögren's syndrome

Mast cells have been linked to rheumatoid arthritis (RA) and are essential to the pathogenesis of RA-like disease in a mouse model. We describe a 34-year-old woman who developed Sj?gren's syndrome concurrently with telangiectasia macularis eruptiva perstans (TMEP), a rare form of cutaneous mastocytosis. The patient had sicca symptoms with an abnormal minor salivary gland biopsy and decreased salivary flow, peripheral neuropathy, an 80 pound weight loss, and a macular erythematous rash that exhibited superficial perivascular mast cell infiltrates on biopsy of lesional skin. This case further underscores the link between mast cells and the development of autoimmunity.     

AV-block III in a patient with sarcoidosis mimicking Sjögren's syndrome

A 55-year old woman with a diagnosis of primary Sj?gren's Syndrome suddenly developed AV-block III. A diagnostic procedure finally revealed sarcoidosis with multiorgan involvement.     

Angiocentric T cell lymphoma of the central nervous system in a patient with Sjögren's syndrome

Patients with Sj?gren's syndrome (SS) have an increased risk of developing lymphoproliferative disorders, mainly non-Hodgkin's lymphoma and pseudolymphoma. We describe a young Caucasian woman with a 7 year history of SS, who presented with meningeal signs and symptoms and was found to have non-Hodgkin's lymphoma of the central nervous system.     

Massive cutaneous follicular lymphoid hyperplasia in a patient with the Sjögren syndrome: 7-year follow-up and immunohistochemical study

The Sjögren syndrome often gives rise to complications such as pseudolymphomas and/or mucosa-/skin-associated lymphocytic tumors (MALT/SALT lymphomas). This paper presents a 74-year-old female patient with the Sjögren syndrome complicated by cutaneous plaques/tumors as cutaneous lymphoid hyperplasia over a 7-year follow-up study period. Immunohistochemical examination disclosed B-cell rich lymphoid follicle formation in the skin with the presence of T and B cells and a varied assortment of features to include abundant plasma cell infiltration. Based on the clinical course and histopathological findings, patient condition was considered benign and arises from reaction toward certain unknown microbes.     

Multiple fibrotic lung nodules in a patient with primary Sjögren's syndrome

Evaluation of bilateral lung nodules noted on imaging poses a diagnostic challenge to clinicians as it can have many differentials from benign to malignant causes. It becomes especially critical to identify them right when there are underlying autoimmune conditions and risk factors for infection. However, a thorough investigation can lead to the recognition of rare associations as described below. We present here a 57-year-old woman who was admitted to the hospital with shortness of breath. Imaging with a computed tomography (CT) scan showed that she had 8 bilateral cystic pulmonary nodules with focal areas of ground-glass opacity and mediastinal lymphadenopathy. Fibrobronchoscopy and histopathological studies were done on the right middle lobe lung nodule demonstrated that the lung nodule was fibrotic with reactive inflammation but showed no malignant cells. Upon further detailed history and chart review, it was noted that the patient had a history of dry eyes leading to an autoimmune workup showing positive antinuclear antibodies (ANA), anti-Ro, and anti-La antibodies with no follow-up since then. This lead to the suspicion that these nodules could be related to underlying Sjögren's syndrome. Initial inpatient management with intravenous steroids showed significant improvement in her symptomatology. Hence, we present this rare association of lung nodules with Sjögren's syndrome and its management for awareness of this condition.     

Mucosa-associated lymphoid tissue lymphomas in two patients with rheumatoid arthritis on second-line agents, and secondary Sjögren's syndrome

We report two patients with rheumatoid arthritis and secondary Sj?gren's syndrome whose disease was complicated by a mucosa-associated lymphoid tissue (MALT) lymphoma. Although this particular type of lymphoma is associated with primary Sj?gren's syndrome, it has not been described, to our knowledge, in the context of rheumatoid arthritis and Sj?gren's syndrome. The potential causative factors are discussed.