Primary Light-chain Amyloidosis Featuring Worm-like Small Bowel Polyposis
نویسندگان
چکیده
منابع مشابه
Primary Light-chain Amyloidosis Featuring Worm-like Small Bowel Polyposis
A 66-year-old man with a history of asthma presented with abdominal distension of one month in duration. Computed tomography revealed jejunal wall thickening and distended loops of small bowel (Picture 1). Double-balloon enteroscopy revealed numerous polypoid, worm-like, 1-5 cm masses, while the mucosa in the duodenum and ileum had a normal appearance (Picture 2, 3). One of the masses was remov...
متن کاملLight 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 کاملTreatment of immunoglobulin light chain (primary or AL) amyloidosis.
Not all forms of amyloidosis are systemic. Some patients may present with a localized form and should not be treated with chemotherapy. Some patients with systemic amyloidosis may have secondary, familial, or dialysis-related types.These types are not responsive to chemotherapy. Immunoglobulin light chain (primary or AL) amyloidosis is a plasma cell dyscrasia. Suppression of light chain product...
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 کاملLight-chain (AL) amyloidosis: diagnosis and treatment.
Light-chain (AL) amyloidosis is the most common form of systemic amyloidosis and is associated with an underlying plasma cell dyscrasia. The disease often is difficult to recognize because of its broad range of manifestations and what often are vague symptoms. The clinical syndromes at presentation include nephrotic-range proteinuria with or without renal dysfunction, hepatomegaly, congestive h...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Internal Medicine
سال: 2017
ISSN: 0918-2918,1349-7235
DOI: 10.2169/internalmedicine.8812-17