- 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 oedema 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.
Last updated: Mar 28, 2013