Impact of time to diagnosis on Mayo stages, treatment outcome, and survival in patients with AL amyloidosis and cardiac involvement.
Eur J Haematol
; 107(4): 449-457, 2021 Oct.
Article
em En
| MEDLINE
| ID: mdl-34185342
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.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Protocolos de Quimioterapia Combinada Antineoplásica
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Diagnóstico Tardio
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Tempo para o Tratamento
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Amiloidose de Cadeia Leve de Imunoglobulina
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Insuficiência Cardíaca
Tipo de estudo:
Diagnostic_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Adult
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Aged
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Aged80
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
Eur J Haematol
Assunto da revista:
HEMATOLOGIA
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Alemanha