Researchers have identified factors that predict long-term outlook for people with AL amyloidosis.
This condition, also known as light chain amyloidosis, is a rare disease involving deposits of abnormal amyloid protein that accumulates in tissues and organs, causing a range of symptoms. Diagnosis involves clinical evaluation with blood tests, tissue biopsies, and imaging.
Now, Dr Vaishali Sanchorawala of Boston University, USA, and colleagues have identified new factors that predict outcomes for patients in advanced cardiac AL amyloidosis.
The team recruited 142 patients with newly diagnosed advanced – stage IIIb – AL amyloidosis. Median follow-up was 60 months.
Patients had a median overall survival of only nine months, and those who died sooner tended to have been diagnosed more than 6 months after symptom onset. They also had a bone marrow plasma cell count of more than 10%, higher troponin I levels (indicating heart damage), and a limited walking speed.
Longer survival was seen in those treated with bortezomib and daratumumab-based regimens. Those who had a good haematologic response one month after treatment had a median survival of 47 months.
Reporting in Blood Advances, the team write: “This study identified factors predictive of treatment outcomes and prognostic for survival in advanced cardiac AL amyloidosis. Early haematologic and cardiac responses during treatment predict longer survival.”
Dr Sanchorawala commented: “While AL amyloidosis is a rare condition, symptoms can mimic those of many other more common diseases, making it difficult for clinicians to diagnose it early.
“By understanding the significance of early treatment responses, we can better guide our approaches to therapy and improve patient outcomes.”
Gustine JN, Staron A, Mendelson L, Joshi T, Gopal DM, Ruberg FL, Siddiqi OK, Sanchorawala V. (2023) “Predictors of treatment response and survival outcomes in patients with advanced cardiac AL amyloidosis.” Blood Advances, doi: 10.1182/bloodadvances.2023010324
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