Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Amyloidosis results from the deposition of amyloid proteins in organ and tissues. It can be localized or systemic. Amyloidosis may be classified as either primary or secondary. Primary is idiopathic, whereas the secondary form is associated with chronic inflammatory or infectious process. Amyloidosis is also related to myeloma multiplex (plasmacytoma) or located as tumor like deposits in isolated organ without systemic involvement. Localized amyloidosis in the head and neck is rare and can have various manifestations. A case of surgically treated localized amyloidosis of the pharynx is presented. The study is focused on case history, results of laryngeal, general and pathological examination of the patient. Current options on the structure of amyloid, making diagnosis, treatment and prognosis in patients with amyloidosis are presented.
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PMID:[Localized amyloidosis of upper respiratory tract]. 1855 8

Hypopharyngeal amyloidosis is rare. Management depends on etiology. We report a case of hypopharyngeal amyloidosis and review the characteristics of this exceptional pathology. A 60-year-old woman with a history of diabetes and chronic cervicalgia consulted for dysphagia and deteriorated general health status, which had been evolving for 2 months. Clinical examination found two ulcerations of the lateral edge of the tongue and right pyriform sinus salivary stasis. Panendoscopy found regular swelling of the posterior wall of the hypopharynx and cervical esophagus. The pyriform sinuses and larynx were normal. Cervical CT and MRI showed thickening of the posterior wall of the hypopharynx. Biopsy found amorphous acellular eosinophil interstitial deposits, shown to be amyloid on Congo red staining, leading to a diagnosis of amyloidosis. Etiological assessment pointed to myeloma. The patient was managed by chemotherapy associating melphalan and prednisone. Evolution at 12 months' follow-up was good. Localized amyloidosis is a rare lesion of the superior aerodigestive tract, predominating in the larynx. Hypopharyngeal involvement is exceptional. Diagnosis is histological. Management depends on etiology. Local treatment is exceptional other than in case of complication. Systemic forms with associated myeloma are of poor prognosis.
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PMID:Hypopharyngeal amyloidosis: A case report. 2082 62

Amyloidosis is a heterogeneous group of disorders and may be classified as systemic or localized on the basis of the distribution of amyloid deposition. Infrequently, the urinary tract and supporting retroperitoneum may be involved, and the imaging findings are nonspecific and diverse. Localized amyloidosis usually involves the bladder and often mimics malignancy. Less frequently, the ureter, renal pelvis, and urethra are involved. The most common findings of amyloid deposition are focal or diffuse wall thickening in the urinary tract with intramural calcification that often results in ureteral obstruction. When the renal parenchyma is involved, patients generally develop nephrotic-range proteinuria, and the kidneys appear atrophic with cortical thinning. In systemic amyloidosis, amyloid may infiltrate the retroperitoneal and pelvic soft tissues, encasing the urinary tract, with diffuse soft-tissue thickening and slowly progressive calcification. In both localized and systemic amyloidosis, amyloid lesions are characteristically hypointense at T2-weighted magnetic resonance imaging. Because myeloma or lymphoma is often present with systemic amyloidosis, biopsy is necessary to diagnose the condition. Amyloid lymphadenopathy characteristically appears as nodal enlargement with calcification and low attenuation at computed tomography. Radiologists should be familiar with the imaging features of amyloidosis that, in the appropriate clinical context, may indicate the diagnosis.
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PMID:Imaging evaluation of amyloidosis of the urinary tract and retroperitoneum. 2199 82