Cases reported "Amyloidosis"

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1/17. Amyloidoma of the nose in a pediatric patient: a case report.

    Localized amyloidosis in the head and neck is a rare and benign disease. larynx is the most common site of involvement and accounts for 0.2% to 1.5% of benign laryngeal tumors. The oral cavity and pharynx may also be involved in localized amyloidosis of the head and neck. There are only 7 cases of localized nasopharyngeal amyloidosis, and 8 cases of localized nasal amyloidosis reported to date. Out of these 8 cases of nasal amyloidosis, only 1 of them is a pediatric patient. We present the second reported case of localized nasal amyloidosis in a pediatric patient. This case report describes a 10-year-old girl with a 1-year history of right-sided nasal obstruction and mucoid discharge. Examination revealed an irregular erythematous, waxy gray mass arising from the right inferior turbinate. Computed tomography and magnetic resonance imaging showed the mass arising from the inferior turbinates, whereas other investigations excluded systemic amyloidosis. histology from surgical excision revealed amyloidosis. This case illustrates that although amyloidoma of the nose is rare, it should also be considered as part of the differentials of a nasal mass even in pediatric patients. [editorial comment: These authors demonstrate that an index of suspicion and confirmation of that suspicion through biopsy and subsequent pathologic evaluations Maybe the only way to avoid missing this potentially important cause of nasal obstruction.]
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keywords = oral cavity, cavity
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2/17. Nasopharyngeal amyloidosis.

    PURPOSE: To discuss the presentation of localized amyloidosis affecting the nasopharynx and discuss the management options. amyloidosis in the head and neck is a rare and benign condition that usually takes the form of localized amyloidosis. Because systemic amyloidosis markedly shortens life expectancy owing to its involvement with vital organs, rectal biopsy or fat aspiration of the anterior abdominal wall must be carried out to exclude systemic involvement. Localized amyloidosis in the head and neck can involve the orbit, sinuses, nasopharynx, oral cavity, salivary glands, and larynx. methods: We present the case of a patient with conductive hearing loss and serous otitis media with effusion secondary to nasopharyngeal amyloidosis, as well as present a review of the literature. RESULTS: Only a few cases of nasopharyngeal amyloidosis have thus far been reported. patients with this disease can also present with recurrent epistaxis, postnasal drip, nasal obstruction, and eustachian tube dysfunction. Localized amyloidosis of the nasopharynx, which is slow growing, has proved difficult to treat because it can persist or recur despite surgical treatment. Furthermore, bleeding may be a major complication in treating patients with nasopharyngeal amyloidosis by transpalatal excision because the amyloid deposits cause vascular wall fragility. Finally, there is no evidence that surgical treatment of nasopharyngeal amyloidosis can prolong survival or that localized amyloidosis can progress to systemic amyloidosis. For these reasons, we elected to treat our patient with a tympanostomy tube and observation. CONCLUSION: In the absence of systemic disease, localized amyloidosis of the nasopharynx may be treated conservatively.
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keywords = oral cavity, cavity
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3/17. Oral involvement in a case of AA amyloidosis.

    A case of oral involvement of amyloid associated (AA) amyloidosis (secondary systemic amyloidosis) in a patient who is suffering from psoriatic arthritis and ankylosing spondylitis, is described. The patient was complaining of pain in the oral cavity (burning mouth), especially on the tongue, and difficulty in chewing and swallowing foods. Oral examination revealed a few papules on the dorsum of the tongue associated with xerostomia. In addition, two slightly painful, small ulcers, localized on the vestibule of the mouth were observed. biopsy of the tongue demonstrated amyloid deposits.
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keywords = oral cavity, cavity
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4/17. Low grade multiple myeloma that presented as a labial swelling -- a case report.

    Myeloma is a disease of the bone marrow in which there is malignant proliferation of plasma cells. Myeloma is usually associated with the accumulation of a monoclonal immunoglobulin or light chains in plasma and subsequently an increase in light chains in the urine (Bence-Jones proteins). Renal failure can occur and bone destruction in the axial skeleton may ensue with pain and fractures. amyloidosis associated with multiple myeloma is a relatively common finding. The most frequently reported oral sign of amyloidosis is macroglossia. There are numerous cases in the literature of amyloid deposition in tongues related to multiple myeloma. However, none of these cases describe amyloid deposition surrounding arteries in the oral cavity. We therefore report a case of an innocuous lower labial swelling, which subsequently led to the diagnosis of amyloid deposition surrounding an artery, the cause of which was later shown to be multiple myeloma.
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keywords = oral cavity, cavity
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5/17. Three cases of the nodular pulmonary amyloidosis with a longterm observation.

    Longterm observation with chest radiograph and computed tomography (CT) scan was performed for pulmonary amyloidosis. There are few reports of primary pulmonary amyloidosis with a longterm observation. We encountered three cases of nodular pulmonary amyloidosis observed by intermittent chest radiograph or CT for 5 years or more. The patients were a 54-year-old man, and 67- and 68-year old women. For diagnosis, transbronchial biopsy and percutaneous lung biopsy were performed. Amyloid nodules grew slowly and two cases showed findings of cavity and calcification.
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keywords = cavity
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6/17. Marginal zone B-cell lymphoma of minor salivary gland representing tumor-forming amyloidosis of the oral cavity. A case report.

    We report here a case of mucosa-associated lymphoid tissue (MALT)-type lymphoma arising from the minor salivary gland of the oral cavity exhibiting tumor-forming amyloidosis. The patient was a 64-year-old Japanese woman who presented with 4-year history of a left soft palate mass. Despite multiple and multifocal recurrences including the lip, soft palate, tongue, oral base and vocal code and soft palate, the tumor remained localized in the upper aerodigestive tract, and the patient did not develop multiple myeloma during the course of disease. Histologically, the majority of the lesion was occupied by amyloid deposition. Only the periphery of the lesion contained numerous plasmacytoid cells, along with occasional centrocyte-like cells. In addition, lymphoepithelial lesion and follicular colonization were noted. The present case indicates that primary minor salivary gland MALT-type lymphoma appears to be the cause of tumor-forming amyloidosis of the upper aerodigestive tract including the larynx.
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keywords = oral cavity, cavity
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7/17. Bullous amyloidosis of the oral cavity: a rare clinical presentation and review.

    Bullous amyloidosis (BA) is a rare cutaneous manifestation primarily of systemic amyloidosis, a disease in which abnormal proteinaceous material is formed and deposited in response to inflammatory conditions and plasma cell dyscrasias. Hemorrhagic bullae indicative of BA are usually associated with purpura and may be the initial clinical signs of systemic amyloidosis or monoclonal gammopathies, such as multiple myeloma and Waldenstrom's macroglobulinemia. Bullous amyloidosis of the oral cavity is highly uncommon and can mimic other vesiculobullous lesions of the oral mucosa. This article highlights an unusual case of oral BA and reviews important aspects of the disease.
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keywords = oral cavity, cavity
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8/17. amyloidosis of the oral cavity: report of five cases.

    Five cases of amyloidosis involving structures in the oral cavity are reported. Three cases appeared to be secondary systemic amyloidosis, one case was the systemic form associated with multiple myeloma, and one case appeared to represent nodular or localized amyloidosis. All cases appeared in persons over 63 years of age, and the most common area of involvement was the tongue. The nature of so-called amyloid deposits in certain odontogenic tumors is discussed in light of recent knowledge of amelogenins and enamelins.
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ranking = 5
keywords = oral cavity, cavity
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9/17. Surgical complications of amyloid disease.

    The case of a man with primary systemic amyloidosis without myelomatosis and long-term survival is described. The patient has had major surgical complications from large amyloid deposits in the colon, dorsal spine and peritoneal cavity. The patient remains well 14 years after diagnosis.
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keywords = cavity
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10/17. Production of monoclonal antibody against amyloid fibril protein and its immunohistochemical application.

    An autopsy case of multiple myeloma (IgA-lambda) with extensive amyloid arthropathy of the systemic joints was described. Heavy deposits of amyloid (amyloidoma) were observed in the articular cavities of the joints. Furthermore, numerous amyloid deposits were found in the walls of small blood vessels in the general organs. Incubation of the paraffin sections of amyloid with potassium permanganate produced little loss of congo red affinity and apple-green birefringence under polarized light. A crude preparation of amyloid protein was isolated from a frozen intra-articular mass, which had been obtained at necropsy, and injected into the footpads of BALB/c mice with complete freund's adjuvant. The immune spleen cells were fused with myeloma cells (P3 X 63-Ag8.653) under the presence of polyethylene glycol. Hybridoma cell lines, producing supernatants which reacted not only with amyloid substances but also with normal human tissues, were omitted from the subjects of recloning, and one hybridoma cell line (Am-1) producing a specific monoclonal antibody (MAb) against the immunized amyloid substance was finally obtained. Using the indirect immunoperoxidase method, the specificity of MAb Am-1 was confirmed in the cryostat sections as well as in the formalin-fixed paraffin sections of various organs of the case from which the crude amyloid protein was obtained and used for immunization. Amyloid deposits in 25 cases with amyloidosis or amyloid deposits were examined with MAb Am-1, and 2 cases showed positive reactivity with Am-1. These 2 cases presented primary amyloidosis and focal amyloid deposits in the oral cavity, respectively; congo red staining of amyloid in these cases showed resistance to treatment with potassium permanganate, as observed in the amyloid of the original case used for immunization. trypsin treatment of the sections resulted in a loss of positive reactivity with MAb Am-1 in all these cases. This result indicates that Am-1 recognizes a substance composed of protein molecules. Furthermore, the immunohistochemical distribution of Am-1 positive reaction was consistent with the histological distribution of substance which could be stained by congo red and showed apple-green birefringence under polarized light. These results have suggested that Am-1 reacts with a portion of amyloid protein which is resistant to treatment with potassium permanganate followed by congo red staining.
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keywords = oral cavity, cavity
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