Cardiac amyloidosis: modern aspects of diagnosis and treatment (clinical observation)

Cardiac amyloidosis is a disease characterized by the deposition of insoluble amyloid protein in the intercellular space. AL-amyloidosis is the most aggressive form of systemic amyloidosis with a wide spectrum of clinical manifestations but most often debuting with congestive heart failure. Long latency period and poor prognosis in decompensated heart failure make diagnosis of cardiac amyloidosis in the early stages of the disease including biochemical and immunochemical urine and blood tests, instrumental methods and the gold standard for detecting amyloid deposits – biopsies taken from various organs and tissues – vitally important. Recently, due to regularly modified chemotherapy regimens, a proteasome inhibitor, bortezomib, used in the treatment for multiple myeloma, prognostic improvement of patients with cardiac AL-amyloidosis has been noted. Heart transplantation for amyloidosis is disputable because it is associated with recurrence of amyloid deposition in the graft and progression of failure of other organs. At the same time successful results of heart transplantation in combination with high-dose chemotherapy and bone marrow transplantation have been obtained which in the future may become an alternative in the treatment of patients with heart failure and amyloidosis. This article reports about a case demonstrating the successful use of high-dose chemotherapy for a patient with cardiac AL-amyloidosis and pronounced symptoms of congestive heart failure.