title: Systemic light-chain amyloidosis presenting with rapid progressive heart failure.
abstract: Systemic amyloid light-chain (AL) amyloidosis is caused by deposition of immunoglobulin light-chain proteins. AL amyloidosis is a rapidly progressive disease that affects multiple organs. Cardiac involvement is frequent (50%) and the median survival is 5 months following diagnosis. Although transthoracic echocardiography can show characteristics findings, further diagnostic modalities are required for diagnosis. The gold standard of the diagnosis is presence of the amyloid in a tissue biopsy specimen. We present here a case of AL amyloidosis with presenting rapid progressive heart failure. Despite improvements in diagnostic and therapeutic modalities, diagnosis is difficult and prognosis is still poor in patients with cardiac involvement as in the present case.
authors.affiliation: Marmara University Faculty of Medicine, Department of Cardiology, Kadıköy, İstanbul, Turkey. drsunbul@yahoo.com.tr
authors.forename: Murat
authors.initials: M
authors.lastname: Sunbul
authors.forename: Erdal
authors.initials: E
authors.lastname: Durmus
authors.forename: Tarik
authors.initials: T
authors.lastname: Kivrak
authors.forename: Fatih
authors.initials: F
authors.lastname: Besiroglu
authors.forename: Fatma
authors.initials: F
authors.lastname: Gerin
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Sari
authors.forename: Bulent
authors.initials: B
authors.lastname: Mutlu
mesh_terms: D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D001706:Biopsy; D018450:Disease Progression; D006333:Heart Failure / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D011379:Prognosis
chemical_list: D007147:Immunoglobulin Light Chains
keywords: heart failure; restrictive cardiomyopathy; systemic amyloidosis