Cases reported "Amyloidosis"

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1/15. 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.
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2/15. Fatal pancreatitis associated with systemic amyloidosis in a rheumatoid arthritis patient.

    We report here a case of severe acute pancreatitis associated with systemic AA amyloidosis in a 69-year-old rheumatoid arthritis (RA) patient. AA amyloid deposition was detected on the walls of small pancreatic arteries and arterioles. The acute pancreatitis was resistant to various interventions, and acute necrotizing pancreatitis and multiple organ failure developed. Although AA amyloidosis in RA patients is rarely complicated with acute pancreatitis, acute pancreatitis in such cases could be severe and intractable and might result in a fatal outcome.
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3/15. Cutaneous lymphatic amyloid deposits in "Hungarian-type" familial transthyretin amyloidosis: a case report.

    Multiple transthyretin (TTR) mutations have recently been identified and implicated in the development of familial systemic amyloidoses, but early diagnosis of these disorders is still largely unresolved. We investigated the presence and tissue distribution of TTR-derived amyloid in skin biopsies of a 59-year-old woman carrying the "Hungarian-type" mutation of TTR (Asp18Gly). Clinical symptoms involved severe central nervous system dysfunction without signs of polyneuropathy, also referred to as the "central form" of TTR-related systemic amyloidosis. skin biopsy was also evaluated as a tool in order to diagnose this type of TTR amyloidosis. biopsy samples were collected from the infra-axillary region. light microscopy using congo red and polarized light was used to diagnose amyloid deposits. Subsequently, electron microscopic analysis was performed to correlate the amyloid deposits with vicinal dermal structures. The amyloid class was determined by means of immunocytochemistry. TTR amyloid was primarily localized to lymphatic microvessels in the present case, whereas arterioles were devoid of TTR amyloid deposits. In addition, the well-known association of TTR amyloid with neural structures along the erector pilorum and around the sebaceous and serosal (sweat) glands was also evident. Electron microscopic analysis of amyloid deposits revealed characteristic amyloid fibrils that were irregular in shape, and exhibited a heterogeneous density and a random deposition pattern. Immunocytochemistry confirmed the cutaneous accumulation of TTR amyloid. In conclusion, amyloid deposits were abundantly present in the skin of a patient with "Hungarian-type" TTR amyloidosis; skin biopsy seems to be appropriate for the diagnosis of this disorder. We showed that besides the erector pilorum, sweat glands and nerve terminals, lymphatic microvessels are also severely infiltrated by TTR amyloid. Whether these pathological alterations can exclusively be found in "Hungarian-type" TTR amyloidosis should still be investigated. If such changes are not specific for the Asp18Gly mutation, they may be considered as diagnostic markers for "central" TTR amyloid disorders.
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4/15. Electronmicroscopic observation of amyloid deposits in the vascular walls of the choroid plexus in systemic amyloidosis.

    This report concerns an ultrastructural study of the vessels of the choroid plexus in 3 cases of systemic amyloidosis, sections of formalin-fixed brains being used. Small-sized arteries and arterioles were mainly affected by deposition of amyloid fibrils. In the arterioles, the amyloid fibrils were often so densely accumulated that they separated individual smooth muscle cells of the media and reached the subendothelial region. The basal surface of the endothelium of such vessels showed frequent invaginations filled with amyloid tufts; some of the tufts displaced the thin cytoplasm of the endothelial cells toward the lumen. Occasionally, tufts of amyloid fibrils were found to be exposed to and to float in the vascular lumen. These observations were made only in severely amyloid-laden vessels. Among the several possibilities regarding the tuft-endothelial relationship, the transendothelial transport of amyloid tufts is most likely, but the possibility of intraendothelial processing of amyloid precursor proteins and their secretion to form fibrils extracellularly cannot be excluded.
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5/15. Systemic AA-amyloidosis related to MPO-ANCA microscopic polyangiitis: a case report.

    We report autopsy findings in an 83-year-old woman with myeloperoxidase-type anti-neutrophil cytoplasmic antibody (MPO-ANCA)-positive microscopic polyangiitis and systemic AA amyloidosis. With a diagnosis of MPO-ANCA-related microscopic polyangiitis, the patient was treated with corticosteroids, but she died of intractable enteritis. autopsy showed inactive vasculitis affecting small arteries in kidney, lung, intestinal tract, and skeletal muscle. Gastrointestinal viscera were thickened, and AA-amyloid was demonstrated in arterioles and surrounding tissues. amyloidosis also involved heart, kidney, gallbladder, pancreas, salivary gland, and subcutis. ANCA-positive microscopic polyangiitis appears to have been the likely cause of this patient's AA-amyloidosis.
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6/15. Coexistence of papular mucinosis and systemic amyloidosis associated with lambda-type IgD paraproteinemia.

    The patient is an obese, 55-year-old woman. She noticed purpura at several sites when she was 49 years old. She visited our clinic with a chief complaint of exertional dyspnea at 51 years of age. physical examination revealed localized edema in the left chest wall and lower abdomen with translucent papules in the center. macroglossia, hemorrhagic macules, loss of axillary and pubic hairs, and goose-egg sized swellings of submaxillary lymph nodes were also found. Laboratory data were within the normal range except an increase of IgD and low ECG voltage. Bone-marrow puncture revealed an increase (27%) of plasma cells with some atypicality. serum immunoelectrophoresis clarified IgD lambda-type paraproteinemia and lambda-type Bence Jones proteinemia. Histologically, edema with an increase in GAG was conspicuous in the circumscribed areas of the middle dermis. Amyloid deposition was clarified in the walls of arterioles in the deep dermis.
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7/15. association light chain deposition disease (LCDD) and amyloidosis. One case.

    Up to now, light chain deposition disease (L.C.D.D.) and amyloidosis have been shown to occur in different individuals. A case of association is described in a 76 year old man with terminal renal failure and normal size kidneys. Percutaneous renal biopsy showed glomerular and peritubular fixation of labeled antikappa light chain serum. Stains for amyloidosis were positive in small vessels. Kappa free chains were found in both serum and urine and the bone marrow showed predominantly kappa-containing plasma cells. By electron microscopy both electron-dense granular deposits and amyloid like fibrils were found in the wall of arterioles and small arteries.
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8/15. Amyloid angiopathy combined with granulomatous angiitis of the central nervous system: report on two patients.

    We report here two cases of isolated angiitis of the central nervous system associated with congophilic angiopathy. The clinical history lasted 9 months in the first patient (65 years old) and 9 years in the second patient (59 years old). It was characterized by progressive intellectual deterioration, increased protein content of the CSF and evidence of focal brain lesions in the CT scan. One patient showed chronic intracranial hypertension. Vascular lesions were limited to the brain and were characterized by granulomatous and necrotizing angiitis of the small leptomeningeal and intracortical vessels. Amyloid deposits were present in large amounts along vascular segments showing vasculitis, in foreign body giant cells, in plaque-like structures surrounding diseased perforating arterioles, along cortical microvessels and in many neuritic plaques. Close proximity and topographic overlap of vasculitis and amyloid changes suggest a possible pathogenetic relationship.
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9/15. amyloidosis of the vitreous. fluorescein angiographic findings and association with neovascularization.

    A case of primary systemic amyloidosis with bilateral asymmetric involvement of the vitreous was followed up throughout a 45-month span, during which two vitrectomy procedures were done on one eye. The progressive nature of this disease was documented by serial fluorescein angiograms and fundus photography. The amyloid seemed to be derived from the retina, at small multifocal areas over arterioles and venules that were otherwise clinically and angiographically normal in appearance. follow-up studies suggested that these focal deposits were actually preretinal, since they disappeared after posterior vitrectomy. Peripheral neovascularization was visible in this case, which has not previously been reported. amyloidosis of the vitreous can mimic numerous other diseases. vitrectomy can be effective in restoring visual acuity, although recurrence may be rapid. The diagnosis is readily made by pathologic examination of the vitreous aspirate.
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10/15. cerebral amyloid angiopathy manifesting as recurrent intracerebral hemorrhage.

    Over a period of eight years, a normotensive woman experienced eight strokelike episodes. Computed tomographic (CT) scans obtained during each of the last seven episodes demonstrated intracerebral lobar hemorrhage. cerebral angiography and contrast-enhanced CT scans demonstrated no underlying abnormality. Our patient had recurrent intracerebral hemorrhage (ICH) with no predisposing factors or dementia. The clinical diagnosis was primary cerebral amyloid angiopathy (CAA). brain biopsy specimens demonstrated light microscopic and ultrastructural evidence of amyloid in cerebral arterioles. We believe that the combined clinical, CT, and ultrastructural changes in this case are unique. Recurrent ICH visualized by CT scanning has diagnostic value in CAA.
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