Amyloid proximal tubulopathy: a novel form of light chain proximal tubulopathy
نویسندگان
چکیده
منابع مشابه
Amyloid proximal tubulopathy: a novel form of light chain proximal tubulopathy
Light chain proximal tubulopathy is a paraproteinemic-related kidney disease most commonly seen in patients with a plasma cell dyscrasia. The classic description is that of proximal tubules with kappa-restricted intracytoplasmic crystals in a patient with a clinical Fanconi's syndrome. Recently, other variants of light chain proximal tubulopathy have been described including those without cryst...
متن کاملLambda Light Chain Crystalline Cast Nephropathy and Proximal Tubulopathy
INTRODUCTION P lasma cell dyscrasias such as multiple myeloma (MM) result from clonal proliferation of plasma cells and subsequent overproduction of Igs, including free light chains. Renal dysfunction is a core manifestation of plasma cell dyscrasias. A broad range of kidney pathologies can occur, including light chain cast nephropathy, monoclonal Ig deposition disease, light chain amyloidosis,...
متن کاملFibrillary inclusions in light chain proximal tubulopathy associated with myeloma
A 66-year-old retired librarian was referred to the nephrology clinic for evaluation of a recent onset of asymptomatic proteinuria identified at a routine hypertension check-up. She had a 15-year history of hypertension and was treated with ramipril and bendrofluomethazide with no history of diabetes. The urinary protein–creatinine ratio was elevated at 819 mg/mmol, with no evidence of glycosur...
متن کاملLight Chain Proximal Tubulopathy: Clinical and Pathologic Characteristics in the Modern Treatment Era.
Light chain proximal tubulopathy (LCPT) is characterized by cytoplasmic inclusions of monoclonal LC within proximal tubular cells. The significance of crystalline versus noncrystalline LCPT and the effect of modern therapies are unknown. We reported the clinical-pathologic features of 40 crystalline and six noncrystalline LCPT patients diagnosed between 2000 and 2014. All crystalline LCPTs were...
متن کاملLoss of tubular creatinine secretion as the only sign of tubular proximal cell dysfunction in light chain proximal tubulopathy
Light chain proximal tubulopathy (LCPT) is a rare disease, characterized by cytoplasmic inclusions of light chain (usually kappa) immunoglobulins. Clinical presentation is usually a Fanconi syndrome. The proximal tubular dysfunction can be incomplete, and exceptional cases of LCPT without any tubular dysfunction have even been described. Here, we report a case of LCPT in which the only sign of ...
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: Clinical Kidney Journal
سال: 2012
ISSN: 2048-8505,2048-8513
DOI: 10.1093/ckj/sfs004