Of 34 patients with primary amyloidosis seen at the University of Arizona between 1973 and 1984, 25 had amyloidosis in association with overt multiple myeloma. Response to treatment was evaluable in 16 of the 25 patients. Median survival in the seven patients (44 percent) with response to treatment was 28 months versus a median of seven and a half months for the nine patients without response (p less than 0.001). Although there were no absolute predictors of response, patients with response were much less likely to have cardiac amyloid (14 percent versus 78 percent, p = 0.02), but more likely to have unusually high serum beta 2-microglobulin (above 15 micrograms/dl in three of four with response versus in neither of two without response) and kappa subtype monoclonal protein (57 percent versus 33 percent). Renal amyloid and/or serum creatinine levels above 2 mg/dl in three of seven (43 percent) patients with response did not preclude a good outcome. It is concluded that a chemotherapy induction trial is worthwhile in primary amyloidosis associated with myeloma since a subset of patients can have prolonged survival (more than two years).
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http://dx.doi.org/10.1016/0002-9343(86)90715-1 | DOI Listing |
S D Med
November 2024
Sanford Orthopedics and Sports Medicine, Sioux Falls, South Dakota.
Amyloidosis is a deadly systemic disease in which misfolded proteins accumulate in human tissue eventually leading to morbid dysfunction in multiple organ systems. The prognosis of untreated amyloidosis is poor. Orthopedic manifestations of amyloidosis include carpal tunnel syndrome (CTS), trigger digit, distal biceps tendon rupture, rotator cuff disease, and lumbar spinal stenosis.
View Article and Find Full Text PDFCurr Opin Cardiol
January 2025
Division of Cardiology, The Amyloidosis Center, Knight Cardiovascular Institute, Oregon Health & Science University, Portland, Oregon, USA.
Purpose Of Review: Review advancements in therapies for transthyretin (ATTR-CM) and immunoglobulin light chain (AL-CM) cardiac amyloidosis.
Recent Findings: In ATTR-CM, tafamidis remains the cornerstone therapy, with Food and Drug Administration (FDA) approval for over 5 years. Acoramidis, another transthyretin stabilizer, has very recently been FDA-approved following positive results in the ATTRibute-CM trial.
Background: Light-chain cardiac amyloidosis (AL-CA) is associated with structural and functional changes in the left atrium and left ventricle. This study aims to assess the value of the left atrioventricular coupling index (LACI) assessed by three-dimensional echocardiography (3DE) for predicting primary outcome in AL-CA.
Methods: Participants with biopsy-confirmed AL-CA from April 2022 to February 2024 were prospectively analysed.
Orphanet J Rare Dis
January 2025
Department of Cardiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.
Background: There is no unified prognostic scoring system for light chain cardiac amyloidosis (AL-CA), particularly stage IIIb AL-CA. This study aimed to use invasive haemodynamic information to investigate markers that can more accurately evaluate the prognosis of patients with stage IIIb AL-CA.
Methods: In this retrospective cohort study, we conducted invasive haemodynamic measurements concurrently with myocardial biopsies to diagnose AL-CA.
JACC Cardiovasc Imaging
January 2025
Department of Nuclear Medicine, Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Center for Rare Diseases Research, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Background: Cardiac involvement in amyloid light chain (AL) amyloidosis significantly influences prognosis, necessitating timely diagnosis and meticulous risk stratification.
Objectives: This prospective study aimed to delineate the molecular phenotypes of AL cardiac amyloidosis (AL-CA) by characterizing fibro-amyloid deposition using F-florbetapir and gallium-68-labeled fibroblast activation protein inhibitor-04 (Ga-FAPI-04) positron emission tomography (PET)/computed tomography (CT) imaging. The authors also proposed a novel molecular stratification methodology for prognosis.
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