Improvement of cardiac diastolic function and prognosis after autologous peripheral blood stem cell transplantation in AL cardiac amyloidosis.
نویسندگان
چکیده
AL amyloidosis is a disease in which immunoglobulin L chain is deposited in multiple organs, and the prognosis of cardiac amyloidosis is extremely poor. Although several treatments based on that for multiple myeloma, have been performed, there is no clear evidence that cardiac function is improved. We report a case of AL cardiac amyloidosis with moderate cardiac dysfunction for which we performed autologous peripheral blood stem cell transplantation (auto-PBSCT) in combination with high-dose melphalan therapy. This treatment resulted in significant improvement in cardiac function and good prognosis for about 3.5 years after the diagnosis. Therefore, auto-PBSCT is a possible option as up-front therapy for AL cardiac amyloidosis.
منابع مشابه
Cardiac amyloidosis
Amyloidosis is a family of disorders of the immune system in which one or more organs in the body accumulate amyloid. There are four different forms of amyloidosis: systemic amyloid light chain (AL) amyloidosis, amyloid A (AA) amyloidosis, hereditary, and senile amyloidosis. The abnormal proteins can be found as Bence-Jones proteins in urine mainly in patients with light chain (AL) amyloidosis....
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BACKGROUND AL amyloidosis and multiple myeloma result in extracellular deposition of insoluble fibrillary protein in tissue and organs. Untreated median survival is very poor, and even worse with cardiac involvement. Chemotherapy and peripheral blood stem cell transplantation (PBSCT) have been shown to dramatically improve survival, with hematologic remission documented. Regression of cardiac c...
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ورودعنوان ژورنال:
- Internal medicine
دوره 46 20 شماره
صفحات -
تاریخ انتشار 2007