Kappa light-chain amyloidosis of gastrointestinal tract
نویسندگان
چکیده
منابع مشابه
Light Chain Amyloidosis
Light chain (AL) amyloidosis is caused by a usually small plasma-cell clone that is able to produce the amyloidogenic light chains. They are able to misfold and aggregate, deposit in tissues in the form of amyloid fibrils and lead to irreversible organ dysfunction and eventually death if treatment is late or ineffective. Cardiac damage is the most important prognostic determinant. The risk of d...
متن کاملPrimary systemic amyloidosis and the gastrointestinal tract.
Clinical manifestations of primary amyloidosis can be subtle, particularly when the gastrointestinal tract is involved. We present two cases that illustrate the diversity of such involvement and the subsequent problems with diagnosis. Gastrointestinal effects of primary systemic amyloidosis are reviewed and clinical pointers towards diagnosis are discussed. In particular these cases illustrate ...
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Pneumatosis intestinalis is a radiographic finding of gas pockets within the bowel wall. It can be associated with a range of diagnoses, but the most life-threatening causes are mesenteric ischemia, bowel necrosis, and bowel obstruction. Here we present the case of a patient with multiple myeloma who had pneumatosis intestinalis due to gastrointestinal amyloidosis, which is a rare manifestation...
متن کاملAtypical immunoglobulin light chain amyloidosis
BACKGROUND Primary immunoglobulin light chain amyloidosis (AL amyloidosis) is a plasma cell disorder which mainly affects heart, kidneys, liver, and peripheral nervous system. Cases of atypical AL amyloidosis presented as spontaneous vertebral compression fractures have been rarely reported, and data about the management and clinical outcomes of the patients are scarce. METHODS Herein, we pre...
متن کاملUpdate on treatment of light chain amyloidosis.
Light chain amyloidosis is the most common type of amyloidosis as a consequence of protein misfolding of aggregates composed of amyloid fibrils. The clinical features are dependent on the organs involved, typically cardiac, renal, hepatic, peripheral and autonomic neuropathy and soft tissue. A tissue biopsy or fat aspirate is needed to confirm the presence/type of amyloid and prognostic tools a...
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ژورنال
عنوان ژورنال: Annals of Gastroenterology
سال: 2018
ISSN: 1792-7463
DOI: 10.20524/aog.2018.0319