Another aspect-oriented approach to diagnosis of cardiac amyloidosis.
نویسندگان
چکیده
We read with interest the article by Esterbrook et al on cardiac amyloidosis. Authors demonstrated very excellent clinical presentation and a challenging diagnostic work-up in a patient with cardiac amyloidosis presenting with cough and breathlessness. However, we would like to discuss on another possible straightforward diagnostic approach for this case. A combination of poor R progression on ECG without evidence of coronary artery disease and echocardiographic features of increased ventricular wall thickness in normotensive patients should raise the suspicion of infiltrative heart diseases especially amyloidosis. Urine studies including urine protein to creatinine ratio and urine protein electrophoresis are essential from the fact that proteinuria is present in more than 90% of the cases of amyloidosis. Serum and urine immunofixation can reveal an abnormal monoclonal band in approximately 90% of the cases with primary (AL) amyloidosis as in the case presentation. However, monoclonal protein is present in lower concentration in AL amyloidosis compared with multiple myeloma. So, serum-free light chain assay is a very helpful quantitative test that is abnormal in more than 95% of the cases and should be included in the diagnostic work-up for amyloidosis. An excess of lambda or (less common) kappa from serum-free light chain assay can promptly guide us to the diagnosis of AL amyloidosis.
منابع مشابه
Diagnostic approach to cardiac amyloidosis.
Amyloidosis is a relatively rare disease that may be underdiagnosed and could affect the entire human body. Many organs may be affected, which could increase the morbidity and mortality. Cardiac involvement is the leading cause of poor prognosis. Patients with cardiac amyloidosis are usually admitted with heart failure. The clinical presentation varies greatly, and using the correct approach is...
متن کامل[Familial approach in hereditary transthyretin cardiac amyloidosis].
Cardiac amyloidosis is a disease of complex diagnosis and treatment. Some subtypes of cardiac amyloidosis are inherited. Among these, the most common variant is caused by mutations in the transthyretin gene. Correct identification of amyloidosis produced by a genetic defect is of great importance because it modifies the diagnostic and therapeutic approach in patients and their families. We desc...
متن کاملA Rare Presentation of Multiple Myeloma: A Case Report of Hepatic Amyloidosis
The clinically reported case of liver involvement with multiple myeloma (MM) is rare. Amyloidosis, defined as a tissue deposition of clonal light-chain fibrils, has been reported in 10-15% of the MM patients. We described a rare MM patient with the primal presentation of fulminant hepatic failure and biliary system involvement due to amyloidosis. Our patient had the primal symptoms of hyperbili...
متن کاملCardiac amyloidosis: An update on diagnosis and treatment.
Cardiac amyloidosis (CA), once thought to be a rare disease, is increasingly recognized due to enhanced clinical awareness and better diagnostic imaging. CA is becoming of heightened interest to the cardiology community given more effective treatment strategies for light chain amyloidosis (AL), as well as emerging therapies for transthyretin amyloidosis (ATTR). Furthermore, reversing amyloid de...
متن کاملNew tools to diagnose and follow FAC patients: biomarkers
In the last decade, the availability of the cardiac biomarkers N terminal pro-natriuretic peptide type-B (NT-proBNP) and troponins has radically changed the approach to diagnosis, staging, and response assessment of cardiac amyloidosis. Despite similar appearance at standard imaging, the main types of amyloidosis involving the heart are characterized by different rates of progression and differ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Thorax
دوره 68 9 شماره
صفحات -
تاریخ انتشار 2013