title: Clinical profile and treatment outcome of older (>75 years) patients with systemic AL amyloidosis.
abstract: Systemic AL amyloidosis, a disease with improving outcomes using novel therapies, is increasingly recognized in the elderly but treatment and outcomes have not been systematically studied in this group of patients in whom comorbidities and frailty may compound morbidity and mortality. We report the outcomes of 295 patients with systemic AL amyloidosis ≥75 years seen at the UK National Amyloidosis Centre from 2005-2012. The median age was 78.5 years. The median overall survival was 20 months. Two hundred and thirty-eight patients received chemotherapy and 57 elected for supportive care only (overall survival - 24 and 8.4 months, respectively). On intention-to-treat analysis, 44% achieved a hematologic response including a very good partial response or better in 23%. The median overall survival was 6.2 years in patients achieving very good partial response or better at the 6-month landmark analysis and 1.5 years in non-responders. Factors independently indicating a poor prognosis were: cardiac involvement, performance status ≥2; systolic blood pressure <100 mmHg and, on landmark analysis, achieving less than a very good partial response. Treatment of systemic AL amyloidosis in the elderly is challenging. Deep clonal responses are associated with excellent survival and organ responses. Achieving a response to the first-line regimen appears particularly important as outcomes of non-responders are similar to those of untreated patients. Prospective trials with lower toxicity, outpatient treatment regimens are needed.
authors: Center for Amyloidosis and Acute Phase Proteins, University College London (Royal Free Campus), UK.
authors: Sajitha
authors: S
authors: Sachchithanantham
authors: Department of Haematology, Heatherwood and Wexham Park Hospitals, NHS Foundation Trust, UK.
authors: Mark
authors: M
authors: Offer
authors: University of Alberta, Cross Cancer Institute, Edmonton, AB, Canada.
authors: Christopher
authors: C
authors: Venner
authors: Center for Amyloidosis and Acute Phase Proteins, University College London (Royal Free Campus), UK.
authors: Shameem A
authors: SA
authors: Mahmood
authors: Center for Amyloidosis and Acute Phase Proteins, University College London (Royal Free Campus), UK.
authors: Darren
authors: D
authors: Foard
authors: Center for Amyloidosis and Acute Phase Proteins, University College London (Royal Free Campus), UK.
authors: Lisa
authors: L
authors: Rannigan
authors: Center for Amyloidosis and Acute Phase Proteins, University College London (Royal Free Campus), UK.
authors: Thirusha
authors: T
authors: Lane
authors: Center for Amyloidosis and Acute Phase Proteins, University College London (Royal Free Campus), UK.
authors: Julian D
authors: JD
authors: Gillmore
authors: Center for Amyloidosis and Acute Phase Proteins, University College London (Royal Free Campus), UK.
authors: Helen J
authors: HJ
authors: Lachmann
authors: Center for Amyloidosis and Acute Phase Proteins, University College London (Royal Free Campus), UK.
authors: Philip N
authors: PN
authors: Hawkins
authors: Center for Amyloidosis and Acute Phase Proteins, University College London (Royal Free Campus), UK a.wechalekar@ucl.ac.uk.
authors: Ashutosh D
authors: AD
authors: Wechalekar
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D015996:Survival Rate