1/75. Hepatocellular carcinoma with fibrolamellar pattern in a patient with autoimmune cholangitis.A 75-year-old woman with a 15-year history of autoimmune cholangitis underwent orthotopic liver transplantation because of progressive liver decompensation. A clinically unsuspected hepatocellular carcinoma was found. A portion of the tumor showed fibrolamellar differentiation. Hepatocellular carcinoma, either with the usual pattern or with a fibrolamellar pattern, is rare in the setting of primary biliary cirrhosis, but has been seen in the setting of autoimmune hepatitis. Autoimmune cholangitis is a relatively recently recognized form of autoimmune liver disease whose association with hepatocellular carcinoma has yet to be determined.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
2/75. Autoimmune atrophic gastritis with hypergastrinemia.Elevation in fasting serum gastrin levels was found in three patients being evaluated for persistent upper abdominal pain without radiographic evidence of peptic ulcer disease. Fiberoptic endoscopy of the upper gastrointestinal tract in each patient revealed characteristic changes of chronic atrophic gastritis. Gastric biopsies showed diffuse chronic inflammation in the lamina propria, a decrease in the number of parietal cells, and "intestinalization" of gastric mucosa. Total achlorhydria was demonstrated after a maximal histalog stimulus; however, serum levels of vitamin B12 and schilling test values were normal in all three patients. Parietal cell antibodies were found in the serum in all patients in a dilution of 1:20 to 1:80. These cases represent autoimmune (type A) chronic atrophic gastritis and should be distinguished from chronic simple (type B) gastritis, in which serum gastrin levels are normal and no parietal cell antibodies are found in the serum. patients with autoimmune gastritis should be observed at frequent intervals for the occurrence of pernicious anemia or gastric carcinoma.- - - - - - - - - - ranking = 0.14285714285714keywords = carcinoma (Clic here for more details about this article) |
3/75. Antiepiligrin (laminin 5) cicatricial pemphigoid associated with an underlying gastric carcinoma producing laminin 5.Although bullous pemphigoid and cicatricial pemphigoid are sometimes associated with malignancy, it remains uncertain whether such an association is pathogenetically related or just a coincidence attributable to the advanced age of the patients. We report a 61-year-old patient with antiepiligrin (laminin 5) cicatricial pemphigoid (AeCP) associated with an advanced gastric carcinoma. The gastric carcinoma cells in this patient were shown to produce laminin 5 by immunofluorescence microscopy, and the patient's serum contained autoantibodies directed against laminin 5 on immunoprecipitation. Furthermore, the blistering symptoms and the titre of antibasement membrane zone antibodies coordinately changed with the resection and subsequent relapse of the gastric cancer. These observations suggest that the gastric carcinoma producing laminin 5 may have induced the production of autoantibodies to this laminin, which were pathogenic to the skin and mucous membranes in this patient. This report demonstrates a link between this autoimmune subepithelial blistering disease and malignancy. It is of interest and potential great importance to examine other cases of AeCP for such a potential association.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
4/75. sarcoidosis in a patient with linear IgA disease.We report a patient with four conditions in association with linear IgA disease (LAD), only three of which have been reported previously; these latter are ulcerative colitis, autoimmune thyroid disease and carcinoma of the colon, although the carcinoma may have been caused by the ulcerative colitis in this case. Recently, our patient also presented with respiratory symptoms and was found to have sarcoidosis as well, a previously unreported association of this autoimmune bullous disorder. The aetiology of this development may be related to the patient's HLA status or possibly to his treatment with the immunosuppressive agent cyclophosphamide; it is also possible that it is coincidental.- - - - - - - - - - ranking = 0.28571428571429keywords = carcinoma (Clic here for more details about this article) |
5/75. Bazex syndrome mimicking a primary autoimmune bullous disorder.Bazex syndrome is a paraneoplastic condition that is most frequently associated with squamous cell carcinoma of the upper aerodigestive tract. The lesions affect acral areas of the skin, including hands, feet, ears, nose, and, to a lesser extent, elbows and knees. Lesions mimic psoriasis and dermatitis. paronychia and nail dystrophy are frequent. Bullous lesions have been reported only rarely. We report a patient with Bazex syndrome with predominantly bullous lesions that mimicked a primary autoimmune bullous disorder.- - - - - - - - - - ranking = 0.14285714285714keywords = carcinoma (Clic here for more details about this article) |
6/75. Spontaneously occurring neutralizing antibodies against granulocyte-macrophage colony-stimulating factor in patients with autoimmune disease.There is increasing evidence that spontaneous anticytokine autoantibodies are associated with chronic infections and autoimmune diseases. We report the sporadic occurrence in autoimmune diseases of such autoantibodies to granulocyte-macrophage colony-stimulating factor (GM-CSF), a cytokine involved in inflammation and the regulation of proliferation, differentiation and function of granulocytic and monocytic cell lineages. In 41 of 425 patients tested, we found low to moderate levels of autoantibodies binding to GM-CSF in serum or plasma. These were most prevalent in patients with myasthenia gravis (MG). However, neutralizing autoantibodies against GM-CSF were very rare, being found in only three patients. Two had autoimmune MG, one with thymoma (Patient A) and the other (Patient B) with 'seronegative' MG, i.e. without the antiacetylcholine receptor autoantibodies characteristic of most MG patients, and a third (Patient D) had multiple sclerosis. Only very limited amounts of Patient A and Patient D serum/plasma were available for analysis and therefore further studies were carried out on the more plentiful samples from Patient B. The anti-GM-CSF autoantibodies of Patient B were predominantly polyclonal immunoglobulin g and strongly neutralized recombinant human (rh) GM-CSF derived from different expression systems. They had similar immunological and immunochemical characteristics to anti-GM-CSF antibodies that developed in immunocompetent colorectal carcinoma patients following (rh)GM-CSF therapy. In serial samples from Patient B, the anti-GM-CSF autoantibodies were undetectable from diagnosis at age 8 years until at least age 13, but then developed spontaneously during (temporary) withdrawal of immunosuppressive treatment. Their neutralizing activity has persisted since their first detection at age 15 years 1 month, and was at its highest level recently at age 17 years 7 months. There was no obvious association with other autoimmune phenomena, nor were any haematological deficiencies overtly manifested, suggesting that any loss of GM-CSF function may have been compensated for by other cytokines.- - - - - - - - - - ranking = 0.14285714285714keywords = carcinoma (Clic here for more details about this article) |
7/75. Various extrahepatic manifestations caused by hepatitis c virus infection.It has been reported that hepatitis c virus (HCV) causes not only liver disease but also disorders of other organs and tissues. Previously, many HCV-related extrahepatic manifestations have been reported. In this study, we report 2 patients in whom tongue cancer was detected during the treatment of HCV-related liver disease. In one patient, tongue cancer was detected during the treatment of HCV-related liver cirrhosis, and articular rheumatism developed thereafter. The duration of HCV-related liver disease was 10 years. In the other patient, tongue cancer was detected during the treatment of HCV-related hepatocellular carcinoma. This patient had a past history of thyroid disease. The duration of HCV-related liver disease was 6 years. In these patients, the possibility that several conditions incidentally and concurrently developed cannot be denied. However, the conditions described above may be regarded as HCV-related extra-hepatic manifestations. In patients with HCV infection, it is important to examine conditions in organs other than the liver. Careful follow-up is needed.- - - - - - - - - - ranking = 0.14285714285714keywords = carcinoma (Clic here for more details about this article) |
8/75. Autoimmune cytopenias associated with malignancies and successfully treated with intravenous immune globulins: about two cases.The authors report on the cases of two adult male patients presenting with autoimmune cytopenias associated with malignancies: a case of autoimmune haemolytic anemia occurring after remission of Hodgkin's disease and a case of autoimmune neutropenia in the setting of renal carcinoma. High-dose intravenous immune globulins (IIG) administered after failure of corticosteroid therapy produced a rapid and long-lasting response. These cases illustrate that intravenous immunoglobulins may be helpful in refractory cases of autoimmune cytopenias. The association of IIG and corticosteroid could be synergistic and effective independently of the outcome of the underlying disease. The pathophysiogenic mechanisms and literature are discussed briefly.- - - - - - - - - - ranking = 0.14285714285714keywords = carcinoma (Clic here for more details about this article) |
9/75. linear iga bullous dermatosis in a patient with renal cell carcinoma.linear iga bullous dermatosis (LABD) is an autoimmune subepidermal bullous disease with heterogeneous clinical manifestations, characterized by linear deposition of IgA along the epidermal basement membrane zone. We report a patient with a metastasized renal cell carcinoma who developed an extensive blistering eruption. The lesions showed immunopathological findings characteristic of LABD. The patient showed a fair response to prednisolone and dapsone. Treatment to control the LABD was no longer required when interferon-alfa was started as palliative therapy for the metastasized renal cell carcinoma. The association of LABD and malignancies has been documented before and is not due to mere chance alone.- - - - - - - - - - ranking = 0.85714285714286keywords = carcinoma (Clic here for more details about this article) |
10/75. Inflammatory pseudotumor of the submandibular gland: report of a case presenting with autoimmune disease-like clinical manifestations.We report a rare case of inflammatory pseudotumor arising in the submandibular gland, which presented with autoimmune disease-like clinical manifestations. A 70-year-old Japanese man developed masses in both submandibular regions. Laboratory tests revealed polyclonal hypergammaglobulinemia, high titers of antinuclear antibody, and a positive thyroid test. Histologically, the lesion was composed of multiple nodules separated by thick fibrous bands and contained a few atrophic lymphoid follicles and residual ductal structures. At higher magnification, the nodules contained numerous mature plasma cells mixed with myofibroblasts, lymphocytes, and histiocytes. Occasionally, the myofibroblasts were arranged in poorly formed fascicles and in a storiform pattern. polymerase chain reaction analysis failed to demonstrated the rearrangement of the immunoglobulin heavy-chain gene. The patient was free of disease after 72 months follow-up. Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type arising from salivary glands occasionally showed prominent plasma cell differentiation. The present case indicates that inflammatory pseudotumor should be added to the list of different diagnoses for mucosa-associated lymphoid tissue-type lymphoma of the salivary glands.- - - - - - - - - - ranking = 0.066456902185606keywords = ductal (Clic here for more details about this article) |
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