Figure 3.
Supportive therapy in AL amyloidosis. Supportive measures have a fundamental role in the management of AL amyloidosis, with the goal of improving quality of life, relieving symptoms, and sustaining organ function while anti-PC therapy is delivered and takes effect. The mainstay of supportive treatment is diuretic therapy. However, in amyloidosis, cardiac function is preload dependent, and it is important to avoid reduction of intravascular volume. Angiotensin-converting enzyme inhibitors are generally poorly tolerated because of hypotension: they should be used at the lowest possible dose and discontinued if not well tolerated. Transplantation of the organs involved by amyloidosis may render patients with advanced disease eligible for aggressive specific treatment. The main concerns with organ transplantation are occurrence of amyloidosis in the graft and progression in other organs. However, the availability of effective anti-PC treatments allows for consideration of heart transplantation followed by effective chemotherapy in young patients with isolated severe cardiac involvement. Patients who have advanced, irreversible organ damage, despite achievement of complete HR, can also be considered for transplantation of the organs involved. However, early reports still awaiting confirmation suggest that patients who fail to attain organ response despite having achieved complete HR may have persistent minimal residual clonal disease. In these subjects, further chemotherapy, if deliverable, may lead to minimal residual disease negativity and improvement of organ dysfunction. Implantation of left ventricular assist devices is technically feasible for patients with severe heart failure caused by advanced cardiac amyloidosis, but the possible benefit is unclear.

Supportive therapy in AL amyloidosis. Supportive measures have a fundamental role in the management of AL amyloidosis, with the goal of improving quality of life, relieving symptoms, and sustaining organ function while anti-PC therapy is delivered and takes effect. The mainstay of supportive treatment is diuretic therapy. However, in amyloidosis, cardiac function is preload dependent, and it is important to avoid reduction of intravascular volume. Angiotensin-converting enzyme inhibitors are generally poorly tolerated because of hypotension: they should be used at the lowest possible dose and discontinued if not well tolerated. Transplantation of the organs involved by amyloidosis may render patients with advanced disease eligible for aggressive specific treatment. The main concerns with organ transplantation are occurrence of amyloidosis in the graft and progression in other organs. However, the availability of effective anti-PC treatments allows for consideration of heart transplantation followed by effective chemotherapy in young patients with isolated severe cardiac involvement. Patients who have advanced, irreversible organ damage, despite achievement of complete HR, can also be considered for transplantation of the organs involved. However, early reports still awaiting confirmation suggest that patients who fail to attain organ response despite having achieved complete HR may have persistent minimal residual clonal disease. In these subjects, further chemotherapy, if deliverable, may lead to minimal residual disease negativity and improvement of organ dysfunction. Implantation of left ventricular assist devices is technically feasible for patients with severe heart failure caused by advanced cardiac amyloidosis, but the possible benefit is unclear.

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