1/15. Simultaneous phenotypically distinct but clonally identical mucosa-associated lymphoid tissue and follicular lymphoma in a patient with sjogren's syndrome.A 44-year-old woman with a 12-year history of sjogren's syndrome (SS) developed a low-grade mucosa-associated lymphoid tissue (MALT) lymphoma in the parotid gland. Two years later, she presented with generalized lymphadenopathy and hepatosplenomegaly and a follicular lymphoma was diagnosed. To investigate the relationship of the two histologically distinct lymphomas, we re-examined their histology and immunophenotype and studied the lymphomatous tissue from the parotid, cervical lymph node, and spleen using molecular genetic methods. Histologic and immunophenotypic studies confirmed the previous diagnoses and also identified a previously unnoticed focus of follicular lymphoma in the second parotid gland biopsy. polymerase chain reaction (PCR) amplification of the rearranged Ig heavy-chain gene showed the same sized dominant product in the MALT lymphoma and the follicular lymphoma. Similarly, PCR analysis of the t(14:18) translocation yielded an identical sized band from both MALT and follicular lymphoma. Cloning and sequencing of the Ig PCR products showed an identical CDR3 sequence from each lesion, indicating a common clonal lineage. The follicular lymphoma of the parotid gland lymph node and the follicular lymphoma of the spleen showed an identical mutation signature to that of the salivary gland MALT lymphoma. We propose that follicular lymphoma in the parotid gland lymph node may have resulted from colonization of lymphoid follicles by MALT lymphoma cells, following which the tumor cells were induced to express a follicular lymphoma phenotype, due to Bcl-2 overexpression caused by t(14;18), leading to a change in clinical behavior resulting in rapid widespread dissemination of disease. These observations suggest that the distinct phenotypes of low-grade B-cell lymphomas may be the consequence of interplay between genetic and local microenvironmental factors.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/15. sjogren's syndrome, cavitating lung disease and high sustained levels of antibodies to serine proteinase 3.A case is described involving sjogren's syndrome, high sustained levels of antibodies to serine proteinase 3, and cavitating lung disease. Possible diagnoses accounting for this unusual combination include a novel association of sjogren's syndrome and Wegener's granulomatosis (suggested by the high and sustained levels of antibodies to serine proteinase 3) or a rare presentation of bronchiolitis obliterans organising pneumonia. Identification of the true nature of the patients illness facilitated more active management and a swift resolution of the clinical problem.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/15. Primary extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type in the CNS.Extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type has been reported in various internal organs. Here a case is reported of MALT lymphoma developing in the cerebellopontine (CP) angle in a patient with Sjogren syndrome, and the concept of MALT lymphoma of the CNS is introduced. Pathologically, the tumor showed inflammatory features of reactive lymphocytic infiltration with follicle formation, but there were slightly atypical lymphocytes and plasmacytes with B-cell markers. These cells invaded reactive follicles, showing follicular colonization, and showed aberrant expression indicating their neoplastic nature. A review of the literature revealed eight cases of MALT lymphoma originating from the dura mater and one from the CP angle. The average age of patients was 50 years (range 28-66 years), and all patients were female. The tumors were slow to develop and the patients were cured after surgical removal and/or additional therapies. It is proposed that MALT lymphoma should be considered as a differential diagnosis of inflammatory pseudotumor of the CNS.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/15. Low grade marginal zone B cell lymphoma of the breast associated with localised amyloidosis and corpora amylacea in a woman with long standing primary Sjogren's syndrome.Primary low grade marginal zone B cell lymphoma (MZL) of the breast and localised mammary amyloidosis are exceedingly rare entities. This report describes the case of a woman with long standing sjogren's syndrome presenting with asymptomatic MZL of the breast showing plasmacytic differentiation, associated with local ductular amyloidosis. The lesion was discovered incidentally in breast tissue resected for microcalcifications. immunohistochemistry revealed kappa light chain restriction, supporting the neoplastic nature of the infiltrate. A retrospective molecular study of the salivary gland biopsy showed a B cell clone. This is the first report of the association of human mammary ductular amyloidosis with cartwheel shaped material identical to corpora amylacea, usually seen in brain, lung, and prostate, but unknown in the human breast. The excellent outcome without treatment seen in this patient further emphasises the need to distinguish between MZL with plasmacytic differentiation and extramedullary plasmacytoma.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/15. Inclusion body myositis in connective tissue disorders: case report and review of the literature.We report a patient with systemic lupus erythematosus (SLE) and secondary sjogren's syndrome (SS) who developed inclusion body myositis (IBM) which, contrary to the typical presentation of this disorder, was symmetrical in nature although the diagnosis was only made after electron microscopy was performed. Therapy with increased doses of methotrexate proved to be beneficial, with the patient having full recovery after 8 months of therapy. It appears that a subset of IBM may be related to autoimmune disorders, an issue that was disputed in the past, and these patients may have a better prognosis than typical IBM patients. This is the first case report of IBM in a patient who had the dual diagnosis of SLE and SS.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/15. Subacute cutaneous lupus associated with Hashimoto's thyroiditis.Subacute cutaneous lupus erythematosus is a widespread, non-scarring, photosensitive form of histologically specific cutaneous LE. These patients frequently have mild systemic illness marked by musculoskeletal complaints and characteristic serologic abnormalities. Hashimoto's thyroiditis coexists with other diseases of presumed autoimmune nature, including systemic lupus erythematosus. The association between subacute lupus and Hashimoto's disease has not been described. We describe here a patient with Hashimoto's thyroiditis and sjogren's syndrome who developed subacute cutaneous lupus two years later.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/15. dapsone is an effective therapy for the skin lesions of subacute cutaneous lupus erythematosus and urticarial vasculitis in a patient with C2 deficiency.The deficiency of second component of complement (C2d) is the most common hereditary complement deficiency. patients with C2 deficiency are frequently associated with an auto-immune disease process, in particular, systemic lupus erythematosus (LE)-like syndrome and/or vasculitic syndrome or bacterial infections. C2d has been associated with the LE subset of subacute cutaneous LE (SCLE), the presence of anti-Ro (SSA) antibodies, and the human leukocyte antigen (HLA) types A10, B18, DR2. We describe the clinical, serologic and immunogenetic data in a patient with manifestations of sjogren's syndrome who developed urticarial vasculitis and photosensitive annular SCLE which were effectively treated with oral dapsone. Our case illustrates the dynamic nature of LE.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/15. sjogren's syndrome-like illness associated with the acquired immunodeficiency syndrome-related complex.Three cases of sjogren's syndrome-like illness occurring in patients with the acquired immunodeficiency syndrome (AIDS)-related complex (ARC) are described. All three patients were male. Positive serologic tests for the human immunodeficiency virus (HIV) were documented in two; the third patient was a prison inmate with a history of drug abuse. The lymphoid infiltrate seen in these cases resembled morphologically the features of persistent generalized lymphadenopathy. One patient complained of dry eyes and arthralgias. Autoimmune phenomena including lupus-like anticoagulant, immune thrombocytopenic purpura, and others have been reported in patients with AIDS and ARC. The occurrence of sjogren's syndrome in ARC provides further evidence for autoimmune phenomena arising in the severely damaged immune system of ARC patients. Atypical sjogren's syndrome now requires evaluation for ARC. Proposed criteria for identifying these patients are as follows: 1) young age (less than 40 years); 2) male sex (less than 10 per cent of non-ARC patients are male); 3) homosexuality or bisexuality, or other high-risk groups for AIDS; 4) generalized lymphadenopathy (also seen in rheumatoid arthritis); 5) negative test for rheumatoid factor despite generalized lymphadenopathy; 6) salivary gland lymphoid infiltrate showing features of persistent generalized lymphadenopathy. patients with such features should be studied for hiv antibodies and other evidence of autoimmune phenomena in order to define more precisely the nature of this new Sjogren's-like illness.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/15. Development of chronic lymphocytic leukaemia in a case of sjogren's syndrome with systemic lupus erythematosus.Persistent lymphocytosis and intermittent fever were found in a 68-yr-old Chinese woman 5 yr after the diagnosis of sjogren's syndrome with systemic lupus erythematosus (SLE). A series of examinations--including virology, bone marrow aspiration and surface markers of lymphocytes--was made to evaluate the nature of the lymphocytosis which had not been found previously. All of the results were consistent with the diagnosis of B-cell chronic lymphocytic leukaemia (CLL). Development of CLL in sjogren's syndrome has seldom been described before and may be added to other malignancies associated with sjogren's syndrome.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/15. Ankylosing spondylitis with subsequent development of rheumatoid arthritis, sjogren's syndrome, and rheumatoid vasculitis.Twelve years after the onset of ankylosing spondylitis, a patient developed severe seropositive rheumatoid arthritis with subcutaneous nodules. This was subsequently complicated by sjogren's syndrome and rheumatoid vasculitis. The literature is reviewed, and the nature of the association between ankylosing spondylitis and rheumatoid arthritis is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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