Cardiac AL Amyloidosis (RCD code: III-3A.2a)
نویسندگان
چکیده
منابع مشابه
Native T1 mapping in ATTR cardiac amyloidosis - comparison with AL cardiac amyloidosis - a 200 patient study
Background Transthyretin amyloidosis (ATTR amyloidosis) is an under diagnosed cause of heart failure with no truly quantitative test. Cardiac involvement is the leading cause of death and influences therapeutic choices. Since new therapies are imminent which aim to treat ATTR amyloidosis the lack of a quantitative test represents a critical step for drug development. In cardiac AL amyloidosis, ...
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 کاملIntracardiac emboli as first presentation of cardiac AL amyloidosis.
Daubert JP, Eldar M, Gold MR, Goldberger JJ, Goldenberg I, Lichstein E, Pitschner H, Rashtian M, Solomon S, Viskin S, Wang P, Moss AJ. Effectiveness of cardiac resynchronization therapy by QRS morphology in the Multicenter Automatic Defibrillator Implantation Trial-Cardiac Resynchronization Therapy (MADIT-CRT). Circulation 2011;123:1061–1072. 14. Fang F, Chan JY, Yip GW, Xie JM, Zhang Q, Fung J...
متن کاملPre-contrast ShMOLLI T1 mapping in cardiac AL amyloidosis
Background Multi-organ disease with cardiac involvement carries a very poor prognosis in Systemic AL Amyloidosis. The risk of nephrogenic systemic fibrosis is a significant obstacle in assessing cardiac status using CMR in patients with systemic AL amyloidosis who have advanced renal failure. Measurement of myocardial T1 values has been limited until now, due to long breathhold times. We have d...
متن کاملEfficacy of bortezomib, cyclophosphamide and dexamethasone in treatment-naïve patients with high-risk cardiac AL amyloidosis (Mayo Clinic stage III).
Bortezomib is an active agent in AL amyloidosis and responses to this drug in combination with cyclophosphamide and dexamethasone are both rapid and deep. Here we present an international, multicenter series of 60 patients with Mayo Clinic stage III cardiac amyloidosis to assess the impact of this regimen in improving outcomes in this poor-risk group. The median follow-up for the entire cohort ...
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ژورنال
عنوان ژورنال: Journal of Rare Cardiovascular Diseases
سال: 2016
ISSN: 2300-5505,2299-3711
DOI: 10.20418/jrcd.vol2no8.246