Last Updated: 2013-03-28
An amyloid tissue deposition disease that may have a primary cause or be secondary to other diseases.
Usually presents with unexplained weight loss, fatigue, and edema resistant to diuretic therapy.
Immunofixation of the serum and urine confirms the presence of monoclonal light chains in primary systemic amyloidosis. Biopsy verification of amyloid deposits is essential.
Treatment includes appropriate management of resulting clinical syndromes, such as nephrotic syndrome, cardiomyopathy, and conduction disorders.
Definitive treatment of primary systemic amyloidosis (AL amyloidosis) includes myeloablative high-dose chemotherapy with stem cell reconstitution in selected patients, or chemotherapy.
Classic periorbital purpura
Morie A. Gertz, MD; courtesy of Mayo Clinic
Epocrates, Inc. All Rights Reserved.
Epocrates iPhone Epocrates Android Epocrates Palm Epocrates Free Download
Epocrates Institutional Sales Epocrates Market Research Epocrates CME Epocrates Mobile Resource Centers Epocrates Clinical Content