1/201. Cholestatic jaundice in two patients with primary amyloidosis: ultrastructural findings of the liver.Two patients with primary amyloidosis (amyloid light chain case) and severe cholestatic jaundice are described. liver biopsy in the preterminal stage demonstrated amyloid deposits in the perisinusoidal space and in portal tracts, and hepatocytes were atrophic because of compression by amyloid fibrils. Ultrastructural findings showed amyloid fibrils not only in Disse's space but also in the sinusoids, and the hepatocyte microvilli facing the amyloid fibrils were spicular. There were aggregates of lysosomal granules in the vicinity of bile canaliculi and some bile canaliculi were dilated with loss of microvilli. Amyloid fibrils in the portal tract compressed bile ductules, causing wide intercellular space and separated basement membranes from their epitheliums. These findings suggested disturbance in transporting not only of essential materials from sinusoids to hepatocytes but also of secretory vesicles into bile canaliculi and leakage of bile juice from small bile ductules in preterminal stage of primary amyloidosis.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
2/201. Pneumatosis intestinalis in association with connective tissue disease.Pneumatosis intestinalis in association with connective tissue diseases is an unusual combination whose pathogenesis is not yet understood. Furthermore, steroid medication, often used to treat these diseases, may itself cause pneumatosis. Three cases of scleroderma, systemic lupus erythematosus, and amyloidosis in association with pneumatosis and without prior steroid therapy are presented. The small vessel occlusive pathologic processes in these diseases may cause focal areas of mucosal ischemia resulting in small, perhaps transient ulcerations that allow gas to enter the gut wall from the lumen.- - - - - - - - - - ranking = 13.520248417938keywords = mucosa (Clic here for more details about this article) |
3/201. intestinal obstruction and gastrointestinal bleeding due to systemic amyloidosis in a woman with occult plasma cell dyscrasia.A 60-year-old woman presented to our hospital with repeated vomiting. Upper gastrointestinal endoscopy revealed a 1 cm diameter ulcer with clean base on the roof of the gastric antrum. Histological examination of gastric biopsies revealed abundant amorphous eosinophilic deposits in the submucosa. congo red stain for amyloid was positive. A barium follow-through study revealed a mass in the jejunum causing incomplete obstruction. urine for bence jones protein was negative. serum protein electrophoresis did not reveal any abnormal band and serum immunoelectrophoresis did not detect any monoclonal immunoglobulin. bone marrow examination, however, revealed an increased proportion of plasma cells. Subsequent immunohistochemical staining demonstrated monoclonal lambda light chains in the marrow plasma cells, thereby confirming a plasma cell dyscrasia. amyloidosis involving the gastrointestinal tract can produce a wide variety of non-specific symptoms and signs. A high index of suspicion is necessary to arrive at an early diagnosis. Management consists of supportive therapy for the gastrointestinal tract as well as treatment of the underlying condition.- - - - - - - - - - ranking = 13.520248417938keywords = mucosa (Clic here for more details about this article) |
4/201. Massive gastrointestinal hemorrhage in a case of amyloidosis secondary to rheumatoid arthritis.A case of a 60-year-old woman with secondary gastrointestinal amyloidosis to rheumatoid arthritis is reported. biopsy findings in the mucosa of the stomach and lower gastrointestinal tract revealed amyloidosis. Endoscopic examination of the lower gastrointestinal tract revealed multiple nodular elevations. The patient showed massive melena. Emergency angiography was performed and an extravasation was found at branches of the jejunal artery. Embolization was performed and this lead to a good prognosis. patients with massive hemorrhages following gastrointestinal amyloidosis generally have a poorer prognosis. Embolotherapy performed for the present case might represent an effective therapeutic method for gastrointestinal hemorrhage in gastrointestinal amyloidosis.- - - - - - - - - - ranking = 13.520248417938keywords = mucosa (Clic here for more details about this article) |
5/201. hepatomegaly and cholestasis as primary clinical manifestations of an AL-kappa amyloidosis.A 53-year-old man, who presented with weight loss over a period of 10 months, hepatomegaly, markedly raised cholestatic enzymes and Ca 19-9, was initially suspected of suffering from metastatic cholangio-carcinoma. liver biopsy revealed depositions of AL-amyloid. Further investigations confirmed a generalized amyloidosis. Biopsies taken from the gastric, colonic, and bronchial mucosa all showed depositions of amyloid. A nephrotic syndrome was interpreted as being secondary to the renal involvement. echocardiography identified changes which were consistent with cardiac involvement. A plasmacytoma or lymphoma was excluded. At the time of diagnosis the patient was in a good physical condition with normal renal function. Within a few weeks the renal function deteriorated and after 2 months the patient developed ascites and became jaundiced. Four months after initial presentation the patient died from cardiac failure.- - - - - - - - - - ranking = 13.520248417938keywords = mucosa (Clic here for more details about this article) |
6/201. Shedding of peripheral cytoplasm - a mechanism of liver cell atrophy in human amyloidosis.A liver biopsy specimen from a case of primary amyloidosis was investigated by electron microscopy. The cytoplasmic periphery of the hepatocytes showed degenerativechanges which are interpreted as indicating shedding of peripheral parts of the cytoplasm. Two main variants of this process could be discerned: 1) Protrusion and sequestration of hernia-like blebs of cytoplasm, and 2) shedding of vesicles derived from degenerated endoplasmic reticulum. In the latter case transient defects of the plasma membrane seem to be relevance. endoplasmic reticulum and cytoplasmic ground substance appeared to be shed preferentially, whereas mitochondria are retained within the cell. As a consequence the fractional volume of the mitochondria in the cytoplasm of atrophic cells is markedly increased. Shedding of peripheral cytoplasm, therefore, seems to be an effective mechanism enabeling the cell to adapt the mass and the composition of its cytoplasm to an unfavourable environment.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
7/201. Primary localized conjunctival amyloidosis presenting with recurrence of subconjunctival hemorrhage.PURPOSE: To report the ocular presentation and histopathology of a patient with primary localized conjunctival amyloidosis. methods: A 38-year-old woman presented with a recurrence of episodes of severe bilateral subconjunctival hemorrhage. Ocular examination revealed yellowish, marked folding and redundancy of the conjunctiva in the inferior cul-de-sac of each eye. RESULTS: After two initial conjunctival biopsies that showed only chronic inflammation, a third biopsy revealed the presence of amyloid in the substantia propria of the conjunctiva. CONCLUSION: Primary localized conjunctival amyloidosis is rare and usually diagnosed histologically instead of clinically. recurrence of subconjunctival hemorrhage may be the initial presentation. Evaluation for systemic diseases is advised, though the results of the examination are almost always negative.- - - - - - - - - - ranking = 28.375035776695keywords = propria (Clic here for more details about this article) |
8/201. Amyloid myopathy masquerading as polymyositis.OBJECTIVE: It is not well appreciated that the clinical presentation of amyloid myopathy can mimic that of polymyositis. By retrospective clinicopathologic analysis we determined distinctive features of amyloid myopathy that differentiate the 2 diseases. methods: Two patients with clinical and histologic evidence of an inflammatory myopathy had fatal outcomes despite appropriate treatment for polymyositis. Their clinical course and original pathologic specimens were reviewed. In addition, original tissue samples were obtained and analyzed using congo red staining and immunoperoxidase. RESULTS: The initial diagnosis of polymyositis was supported in both cases by muscle biopsies showing inflammatory infiltrates and elevations of creatine phosphokinase and by classic electromyography. Retrospective evaluation of the initial muscle biopsies disclosed subtle but incontrovertible evidence of vascular amyloid. Further analysis of the original specimens confirmed the presence of immunoglobin light chain (AL) amyloid. CONCLUSION: Amyloid myopathy can mimic polymyositis. Both can have similar clinical symptoms, as well as inflammatory infiltrates on muscle biopsy. Failure to recognize amyloid myopathy deprives patients of potentially life prolonging treatment. congo red staining and immunohistochemical analysis of tissue could prevent misdiagnosis.- - - - - - - - - - ranking = 28.375035776695keywords = propria (Clic here for more details about this article) |
9/201. The pattern of amyloid deposition in the lung.A review of routine histopathological samples and autopsies examined at the Department of pathology, University of Malaya revealed 15 cases of amyloidosis of the lung. Two were localized depositions limited to the lung while in the remainder, lung involvement was part of the picture of systemic amyloidosis. Both cases of localized amyloidosis presented with symptomatic lung/bronchial masses and a clinical diagnosis of tumour. histology revealed "amyloidomas" associated with heavy plasma cell and lymphocytic infiltration and the presence of multinucleated giant cells. In both cases, the amyloid deposits were immunopositive for lambda light chains and negative for kappa chains and AA protein. One was a known systemic lupus erythematosus patient with polyclonal hypergammaglobulinaemia. The other patient was found to have plasma cell dyscrasia with monoclonal IgG lambda gammopathy. Both patients did not develop systemic amyloidosis. In contrast, lung involvement in systemic AA amyloidosis was not obvious clinically or macroscopically but was histologically evident in 75% of cases subjected to autopsy. Amyloid was detected mainly in the walls of arterioles and small vessels, and along the alveolar septa. It was less frequently detected in the pleura, along the basement membrane of the bronchial epithelium and around bronchial glands. In one case of systemic AL amyloidosis associated with multiple myeloma, an "amyloidoma" occurred in the subpleural region reminiscent of localized amyloidosis. These cases pose questions on (1) whether localized "tumour-like" amyloidosis is a forme fruste of systemic AL amyloidosis and (2) the differing pattern of tissue deposition of different chemical types of amyloid fibrils, with the suggestion that light chain amyloid has a greater tendency to nodular deposition than AA amyloid.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
10/201. Primary bilateral mucosa-associated lymphoid tissue lymphoma of the breast with atypical ductal hyperplasia and localized amyloidosis. A case report and review of the literature.Primary non-Hodgkin lymphoma of the breast is a rare disease. Primary mucosa-associated lymphoid tissue lymphoma is even rarer, and bilateral involvement is exceptional. We describe a case of primary bilateral breast mucosa-associated lymphoid tissue lymphoma with bilateral atypical ductal hyperplasia and bilateral localized amyloidosis in a 64-year-old woman with a history of arthritis and systemic lupus erythematosus and its clinical, histologic, and immunohistochemical features. Microscopic examination of the breast lesion showed dense periductal and perilobular small and plasmacytoid lymphocytes with eosinophilic amyloid in the vessels and the stroma. Bilateral single foci of atypical ductal hyperplasia were also noted. Fine needle aspiration showed small and large lymphocytes and plasma cells. Molecular analysis demonstrated a heavy chain immunoglobulin H gene rearrangement. flow cytometry studies showed an abnormal B-cell population. The combined histologic, paraffin immunohistochemistry, flow cytometry, and molecular results were considered diagnostic for low-grade mucosa-associated lymphoid tissue lymphoma. The patient underwent bilateral local breast radiation without other organ or site involvement.- - - - - - - - - - ranking = 94.641738925567keywords = mucosa (Clic here for more details about this article) |
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