BACKGROUND: AL amyloidosis and multiple myeloma result in extracellular deposition of insoluble fibrillar protein in tissues and organs. Untreated median survival has been documented at 12 months. Cardiac infiltration decreases survival to five months. Chemotherapy and bone marrow transplantation (BMT) have been shown to improve survival when haematological remission is documented. This study aimed to assess if remission could result in cardiac structural improvement. METHODS: 269 patients were treated with BMT for amyloidosis from 1997 to 2010. Cardiac amyloidosis was identified in 30 patients by echocardiographic criteria. Echocardiography was performed before and after BMT. RESULTS: Thirteen of 30 patients with cardiac amyloidosis died during follow-up. No change in cardiac structure was seen in 11 patients. Average survival was 49 months from BMT for non-responders. Fifteen patients had cardiac normalisation (responders). The average time to normalisation was 25 months. Only two responders died. Average survival for responders was 71 months (p < 0.0001 compared with non-responders). Normalisation of cardiac structure was highly predictive of survival (Fisher's exact test p = 0.0025, relative risk 0.18). CONCLUSIONS: Cardiac amyloidosis patients with haematological remission after chemotherapy and BMT may subsequently normalise cardiac structure and function. Normalisation is highly predictive of survival. Crown
BACKGROUND: AL amyloidosis and multiple myeloma result in extracellular deposition of insoluble fibrillar protein in tissues and organs. Untreated median survival has been documented at 12 months. Cardiac infiltration decreases survival to five months. Chemotherapy and bone marrow transplantation (BMT) have been shown to improve survival when haematological remission is documented. This study aimed to assess if remission could result in cardiac structural improvement. METHODS: 269 patients were treated with BMT for amyloidosis from 1997 to 2010. Cardiac amyloidosis was identified in 30 patients by echocardiographic criteria. Echocardiography was performed before and after BMT. RESULTS: Thirteen of 30 patients with cardiac amyloidosis died during follow-up. No change in cardiac structure was seen in 11 patients. Average survival was 49 months from BMT for non-responders. Fifteen patients had cardiac normalisation (responders). The average time to normalisation was 25 months. Only two responders died. Average survival for responders was 71 months (p < 0.0001 compared with non-responders). Normalisation of cardiac structure was highly predictive of survival (Fisher's exact test p = 0.0025, relative risk 0.18). CONCLUSIONS:Cardiac amyloidosispatients with haematological remission after chemotherapy and BMT may subsequently normalise cardiac structure and function. Normalisation is highly predictive of survival. Crown
Authors: Akhil Shukla; David Wong; Julie A Humphries; Benjamin T Fitzgerald; Katrina Newbigin; John Bashford; Gregory M Scalia Journal: CASE (Phila) Date: 2017-04-24