• 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.

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