1/57. amyloidosis presenting as a perforated giant colonic diverticulum.Colonic amyloidosis is frequent, although rarely radiographically evident. The radiological findings are varied and unspecific. We report a case of perforated giant colonic diverticulum (GCD) diagnosed by helical CT in which amyloid deposition was pathologically demonstrated. The patient was a renal transplant recipient who previously had undergone hemodialysis for 10 years. A possible role of amyloidosis in the pathogenesis of this case is discussed. Giant colonic diverticulum must be included in the spectrum of gastrointestinal manifestations of amyloidosis.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/57. A case of hereditary amyloidosis transthyretin variant Met 30 with amyloid cardiomyopathy, less polyneuropathy, and the presence of giant cells.Transthyretin-Met 30 (TTR-Met 30) is a variant of transthyretin and is usually associated with familial amyloid polyneuropathy. It is rare that patients with TTR-Met 30 will primarily develop amyloid cardiomyopathy. This report presents a patient with late-onset TTR-Met 30 who primarily developed amyloid cardiomyopathy, with less amyloid polyneuropathy in the peripheral nervous system than is usually seen. An autopsy was performed, and histological examination revealed many foreign-body giant cells and macrophages in the area of amyloid deposition that was found in nearly all of the organs.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
3/57. Secondary amyloidosis associated with giant cell arteritis/polymyalgia rheumatica.Although giant cell arteritis (GCA) is characterized by chronic inflammation, secondary (AA) amyloidosis appears to be an exceptionally rare complication of this disorder. We describe an 84-year-old man with biopsy proven GCA and polymyalgia rheumatica (PMR) who was found at autopsy to have AA amyloid deposition in numerous organs, 9 years after his diagnosis of GCA. Persistent musculoskeletal symptoms, attributed to refractory PMR during the patient's life, were likely due to AA amyloidosis. This unrecognized complication of GCA/PMR confounded his therapy, leading to excessive treatment with corticosteroids and methotrexate. This case shows that the occurrence of AA amyloidosis should be considered in patients with "refractory PMR" developing after a period of treatment, and that autopsies play a vital role in enigmatic cases.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
4/57. Secondary (AA-type) amyloidosis in patients with polymyalgia rheumatica.Several cases of systemic amyloidosis associated with polymyalgia rheumatica (PMR) or giant-cell arteritis (GCA) have been described. Nevertheless, the type of amyloid deposit has not been characterized in most of them. Here we report on two patients with PMR (one with associated GCA) who developed nephrotic syndrome and end-stage renal failure caused by massive amyloid deposition. Immunohistochemical analysis showed that the amyloid deposits were of AA type (secondary amyloidosis) in both cases.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
5/57. plasmacytoma of the tonsil with AL amyloidosis: evidence of post-fibrillogenic proteolysis of the fibril protein.We report of a 58-year-old Caucasian man who was referred to the University Hospital with a greatly enlarged left tonsil which showed calcifications on computed-tomography scans. Histopathology revealed a plasmacytoma with secondary AL amyloidosis, ossifications, and multinucleated foreign-body-type giant cells. N-terminal sequencing of amyloid-fibril proteins purified from the formalin-fixed tissue showed the presence of two proteins of different size; these were of lambda-light-chain origin (subgroup V), measured approximately 15.2 kDa and 10.5 kDa, and had identical N-terminal ends (YVLTQPP). When the amyloid deposits were immunolabeled with a polyclonal antibody directed against lambda light chain, they showed two staining patterns: some deposits showed intense immunolabeling while others were not immunoreactive. Immunostaining of amyloid was completely absent after protease pre-treatment. Immunoelectron microscopy with gold-labeled secondary antibodies showed staining that was spatially related to amyloid fibrils and suggested that the antibody probably detected the fibril protein. Therefore, our hypothesis in this case is that the different immunostaining patterns are due to a post-fibrillogenic proteolysis of the fibril protein at the C-terminal end of the light chain, as indicated by the presence of two differently sized lambda-light-chain fragments with identical N-terminal ends.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
6/57. 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 = 0.2keywords = giant (Clic here for more details about this article) |
7/57. dialysis-related spinal canal stenosis: a clinicopathological study on amyloid deposition and its AGE modification.Three cases operated for spinal canal stenosis induced by dialysis-related amyloidosis (DRA) were investigated clinicopathologically. Cases were all-male, and had undergone hemodialysis around 20 years. In two cases, cervical plain x-rays showed only minor spondylotic changes. However, magnetic resonance imaging (MRI), myelography, and computed tomography (CT) showed extradural thickness with compression on the cervical spinal cord and cauda equina. In one case cervical x-rays showed typical destructive spondyloarthropathy (DSA), and MRI showed compression myelopathy. Surgical treatment on both cervical and lumbar spine in two cases and on cervical spine only in one case successfully reduced the symptoms. Extradural thickened tissue and ligament flavum obtained during surgery were studied histopathologically and immunostained by using anti-CD68, anti-beta2-microglobulin (beta2m), and anti-advanced glycation end product (AGE) antibody. congo red stain showed diffuse or nodular amyloid deposition, and immunostaining with anti-beta2m and anti-AGE antibodies also demonstrated the same distribution pattern. Thus, beta2m-positive amyloid tissue in the extradural thickness (extradural amyloid deposition) was immunohistochemically demonstrated to be modified with AGE. Inflammatory reaction with histiocytic and giant cell infiltration was also shown around the amyloid tissues. There were CD68-positive cells, and some cells were positive for AGE and beta2m. These findings suggest that beta2m accumulation and inflammatory reaction finally promote destruction of connective tissues. MRI, CT and/or myelography are necessary for diagnosing spinal canal stenosis accompanied by DRA. In conclusion, we propose a more comprehensive concept of dialysis-related spinal canal stenosis, which includes both DSA and myeloradiculopathy induced by extradural thickness.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
8/57. giant cell arteritis "causing" AA-amyloidosis with rapid renal failure.giant cell arteritis (GCA) is a systemic vasculitic disease, which in very rare cases causes inflammatory complications such as secondary amyloidosis. We describe a well-documented case, with a clinically mild course, of biopsyproven giant cell arteritis as the only apparent cause of systemic AA-amyloidosis. The deterioration in renal function due to amyloid deposition occurred rapidly and only a few months after manifestation of giant cell arteritis and was not reversible by steroid treatment. The renal arteries were normal and there was no glomerulonephritis due to giant cell arteritis. This unique case demonstrates that giant cell arteritis with a mild clinical course is closely associated with early-onset severe secondary amyloidosis.- - - - - - - - - - ranking = 0.8keywords = giant (Clic here for more details about this article) |
9/57. Extramedullary plasmacytoma of the parotid gland. Report of a case with extensive amyloid deposition masking the cytologic and histopathologic picture.BACKGROUND: Extramedullary plasmacytomas are uncommon. Although approximately 90% occur in the head and neck region, parotid gland localization is extremely rare. CASE: This report describes fine needle aspiration and histopathologic findings in an extramedullary plasmacytoma arising in the left parotid gland of a 62-year-old man. Aspiration smears showed multiple amorphous clumps of material admixed with ductal epithelial cells, multinucleated giant cells and inflammatory cells rich in plasma cells, suggestive of pleomorphic adenoma. In surgical material, excessive amyloid deposition was observed. Six months later the tumor recurred, and in the second surgical specimen clusters of atypical plasma cells among amyloid deposits was noted. Clinical and laboratory examination excluded multiple myeloma. After local recurrence, radiotherapy was applied. Ten months later the patient was well and without systemic involvement. CONCLUSION: Two points are important. First, in the salivary gland region, because of the focal metachromasia of amyloid with Giemsa stain, amyloid can be confused with the chondromyxoid matrix of pleomorphic adenoma. Second, although localized forms of amyloid tumor exist, one should keep in mind that amyloid may be so abundant that it may mask an underlying plasma cell neoplasm, as in our case.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
10/57. Giant hepatomegaly and portal hypertension in an elderly patient with primary liver amyloidosis: an uncommon clinical occurrence.Primary systemic amyloidosis (AL) is an uncommon disease characterized by the extracellular deposition of a protein with a beta-fibrillar structure, consisting of monoclonal immunoglobulin light chains, lambda or kappa (ratio of lambda to kappa, 3:1). In systemic amyloidosis liver involvement is frequent but it rarely has clinical importance. The massive and localized liver deposition of amyloid, characterized by marked hepatomegaly and portal hypertension without hepato-cellular failure and by a severe prognosis, without systemic involvement, is less frequent. The authors describe an unusual case of primary hepatic amyloidosis with giant hepatomegaly, intrahepatic cholestasis, portal hypertension and splenomegaly, occurred in an elderly patient.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
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