Cholestasis and liver failure with lambda-AL amyloidosis.
نویسندگان
چکیده
منابع مشابه
AL amyloidosis with renal involvement.
Primary (AL amyloidosis) is a systemic disease characterized by an amyloid deposition process in many organs, with unsatisfactory survival of patients. The monoclonal light chains form the fibrils that deposit and accumulate in tissues. Renal involvement is very frequent in AL amyloidosis and could lead to development of nephrotic syndrome followed by the renal failure in many cases. Classic th...
متن کاملAL Amyloidosis
UNLABELLED DEFINITION OF THE DISEASE: AL amyloidosis results from extra-cellular deposition of fibril-forming monoclonal immunoglobulin (Ig) light chains (LC) (most commonly of lambda isotype) usually secreted by a small plasma cell clone. Most patients have evidence of isolated monoclonal gammopathy or smoldering myeloma, and the occurrence of AL amyloidosis in patients with symptomatic multip...
متن کاملGiant hepatomegaly and portal hypertension in an elderly patient with primary liver amyloidosis: an uncommon clinical occurrence.
Primary systemic amyloidosis (AL) is an uncommon disease characterized by the extracellular deposition of a protein with a beta-fibrillar structure, consisting of monoclonal immunoglobulin light chains, lambda or kappa (ratio of lambda to kappa, 3:1). In systemic amyloidosis liver involvement is frequent but it rarely has clinical importance. The massive and localized liver deposition of amyloi...
متن کاملLiver transplantation in a patient with acute liver failure due to sickle cell intrahepatic cholestasis.
BACKGROUND Sickle cell intrahepatic cholestasis is a potentially catastrophic complication of sickle cell anemia Once acute liver failure develops, transplantation is the only option. We describe a patient with sickle cell intrahepatic cholestasis who underwent liver transplantation. METHODS Data were obtained from the chart. Serial hemoglobin S levels were monitored, and measures were taken ...
متن کاملPeritoneal amyloidosis with myopathy in primary systemic (AL) amyloidosis.
To cite: Al-Adhami A, Steiner K, Ellis S. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2016216548 DESCRIPTION We present an unusual case of primary systemic amyloidosis presenting with myopathy and peritoneal amyloid deposition. Peritoneal amyloid is a rare disease with few published cases. An 85-year-old man was referred to rheumatology with walking difficulti...
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ژورنال
عنوان ژورنال: Gut
سال: 1987
ISSN: 0017-5749
DOI: 10.1136/gut.28.7.903