Impact of time to diagnosis on Mayo stages, treatment outcome, and survival in patients with AL amyloidosis and cardiac involvement

Abstract

Objective To study the impact of time to diagnosis on cardiac Mayo stages, treatment outcome, and overall survival. Methods We retrospectively analyzed 77 consecutive patients diagnosed between 2015 and 2020 with AL amyloidosis and cardiac involvement. Medical history was recorded in standardized form with the help of a questionnaire. Results Time from onset of symptoms of cardiac failure to diagnosis was correlated with the severity of cardiac involvement in modified Mayo 2004 and revised Mayo 2012 staging systems (rs = 0.30, 95% CI: 0.07‐0.50, P = .007 and rs = 0.25, 95% CI: 0.01‐0.45, P = .03). Patients with advanced Mayo 2004 stages received reduced‐intensity regimens and had a lower probability to achieve adequate hematologic‐ and cardiac response after first‐line treatment than patients with early stages (rs = 0.28, 95% CI: 0.04‐0.48, P = .01 and rs = 0.72, 95% CI: 0.55‐0.82, P < .0001) and poorer overall survival (P = .0004). Compared with patients diagnosed within the first year, patients diagnosed after 13‐18 or ≥19 months from first symptoms had a 3‐ to 5 times higher risk of dying. Our data indicate that there is a 12‐month window within which the diagnosis of AL amyloidosis needs to be established to avoid early deterioration and death. Conclusions Sensitizing physicians and raising awareness for the disease are crucial for timely diagnosis and may improve the outcome of the disease.

A Importância Do Conhecimento Sobre a Amiloidose Al Para O Diagnóstico Precoce Com Melhor Prognóstico Dos Pacientes

“…Os sintomas da Amiloidose AL geralmente são reconhecidos tardiamente, sendo o diagnóstico feito, em média, 7 meses após o início dos sintomas (SCHULMAN et al, 2020). Além disso, é importante ressaltar que pacientes com o diagnóstico realizado em estágios avançados da doença, apresentam maior risco de morte (OUBARI et al, 2021).…”

Heart transplantation in cardiac amyloidosis

“…Restrictive cardiomyopathy is the worst manifestation of CA, and it is present in 20% of symptomatic patients at diagnosis [2] . There are different variants that involve the heart: primary (AL) amyloidosis and familial (TTR) amyloidosis are the most common forms [3][4][5] .…”

Characteristics and outcomes of heart transplant recipients with a pretransplant history of malignancy

“…A history of pretransplant malignancy (PTM) has been considered as a relative contraindication for HTx because of the potential for recurrence or de novo malignancy development with immunosuppression, which may impact on post HTx survival 2–10 . However, the number of patients with prior malignancy needing HTx is increasing due to improved cancer survival and increasing numbers of effective, yet cardiotoxic cancer treatment regimen 11–21 . The adult heart recipient characteristics for the most recent era (2010 to June 2018) showed that 8.6% had a history of PTM 22 .…”

“…[2][3][4][5][6][7][8][9][10] However, the number of patients with prior malignancy needing HTx is increasing due to improved cancer survival and increasing numbers of effective, yet cardiotoxic cancer treatment regimen. [11][12][13][14][15][16][17][18][19][20][21] The adult heart recipient characteristics for the most recent era (2010 to June 2018) showed that 8.6% had a history of PTM. 22 However, detailed clinical characteristics and long-term posttransplant outcomes of these patients are largely unknown.…”