High-dose therapy with auto-SCT is feasible in high-risk cardiac amyloidosis

Abstract

Cardiac involvement in light-chain amyloidosis (AL) predicts poor prognosis and is associated with higher TRM and morbidity during high-dose therapy and auto-SCT (HDT-ASCT). We studied the outcomes of 30 patients with cardiac amyloidosis undergoing HDT-ASCT at our center between January 1998 and March 2012. The median age of the patients was 53 years (range, 36-74) with a median follow-up of 35 months (range, 0.4-97 months). Twenty-seven patients (90%) had more than one organ involved besides the heart with 37% with cardiac stage ⩾ 3. Melphalan-based conditioning regimen (140-200 mg/m 2 ) was used for HDT-ASCT. One-year TRM is 10%. Three-year OS and EFS from HDT-ASCT was 83% and 56.8%, respectively. Cumulative incidence of relapse at 3 years was 38.5%. Negative factors affecting survival included age 460 years, lack of novel induction therapy and BM plasmacytosis 410%. We conclude that HDT-ASCT is well tolerated in patients with high-risk cardiac amyloidosis and can lead to improved overall outcomes.

Prognosztikai tényezők könnyűlánc-amyloidosisban

“…Itt a 3 éves teljes túlélés 83%-nak adódott, 10%-os transzplantációhoz társuló halálozás (TRM) mellett. Ez arra utal, hogy szívérintettség esetén is eredményes lehet a fenti terápia [30]. Vizsgálták továbbá, hogy gastrointestinalis, perifériás ideg-, máj-és tüdőérintettség formájában megjelenő AL-amyloidosis kezelésében is ugyanolyan hatékony-e a nagy dózisú kemoterápiát követő ASCT, mint a gyakran vizsgált szív-és veseamyloidosisban.…”