signs and symptomspurpuramacroglossiadiagnosisdrug therapytreatmentoutcomeBackground. Systemic amyloidosis is a rare disorder which usually occurs in aged persons and has a poor prognosis. Systemic amyloidosis can be primary, occasionally associated with multiple myeloma, or secondary, associated with another...
The biopsy could injure the brain and while the results might provide a diagnosis, it would be unlikely to change treatment (because there is no effective treatment for amyloid in the brain). When amyloidosis is diagnosed or highly suspected, additional blood and urine tests will be done to ...
For example, primary amyloidosis treatment includeschemotherapy,corticosteroids, and Velcade (bortezomib), a targeted therapy for amyloidosis.22These medicines can slow down organ damage and improve quality of life. Velcade works by targeting processes that lead to the development of abnormal amyloid prote...
and organ dysfunction reversed if the concentration of theamyloidogenic proteinis reduced or zeroed. At present, the most effective approach to treatment of the systemic amyloidoses involves shutting down, or substantially reducing the synthesis of the amyloid precursor, or, as in the case of β2...
Prognosis is affected by the rate of synthesis and the concentration of the circulating precursor. Accurate diagnosis of the underlying disease is mandatory as the treatment is based on disease control and inhibition of amyloid precursor production. Organ-specific treatment, such as transplantation, ...
indicate signs of the amyloid protein, but only bone marrow tests or biopsies of affected tissues or organs can positively confirm the diagnosis. Some tests are done only once to establish a diagnosis, while others are repeated periodically to monitor disease progression and response to treatment. ...
stain of a biopsy specimen. Currently, specimens are obtained by aspiration of subcutaneous abdominal fat. (See Workup.) Current choices for first-line treatment of systemic L-chain amyloidosis are autologous stem cell transplantation (ASCT) and combination chemotherapy (see Treatment and Medication)....
Treatment is the same as with other organ involvement and is targeted at the underlying plasma cell dyscrasia [3, 20]. This patient presented with heart failure with pre- served ejection fraction (HFpEF) and atrial fibrillation; two hallmarks of amyloid cardiac involvement [3, 9]. A past ...
Description of transthyretin S50A, S52P and G47A mutations in familial amyloidosis polyneuropathy. Amyloid. 2013;20(4):221–5. Article PubMed CAS Google Scholar Adams D, Suhr B, Hund E, et al. First European consensus for diagnosis, management, and treatment of transthyretin familial amyloid...