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Impact of time to diagnosis on Mayo stages, treatment outcome, and survival in patients with AL amyloidosis and cardiac involvement.
Oubari, Sara; Naser, Eyad; Papathanasiou, Maria; Luedike, Peter; Hagenacker, Tim; Thimm, Andreas; Rischpler, Christoph; Kessler, Lukas; Kimmich, Christoph; Hegenbart, Ute; Schönland, Stefan; Rassaf, Tienush; Reinhardt, Hans Christian; Jöckel, Karl-Heinz; Dürig, Jan; Dührsen, Ulrich; Carpinteiro, Alexander.
Afiliación
  • Oubari S; Department of Hematology and Stem Cell Transplantation, West German Cancer Center, University Hospital Essen, Essen, Germany.
  • Naser E; Interdisciplinary Amyloidosis Network, University Hospital Essen, Essen, Germany.
  • Papathanasiou M; Institute of Molecular Biology, University of Duisburg-Essen, Essen, Germany.
  • Luedike P; Interdisciplinary Amyloidosis Network, University Hospital Essen, Essen, Germany.
  • Hagenacker T; Department of Cardiology and Vascular Medicine, West German Heart and Vascular Center, University Hospital Essen, Essen, Germany.
  • Thimm A; Interdisciplinary Amyloidosis Network, University Hospital Essen, Essen, Germany.
  • Rischpler C; Department of Cardiology and Vascular Medicine, West German Heart and Vascular Center, University Hospital Essen, Essen, Germany.
  • Kessler L; Interdisciplinary Amyloidosis Network, University Hospital Essen, Essen, Germany.
  • Kimmich C; Department of Neurology, University Hospital Essen, Essen, Germany.
  • Hegenbart U; Interdisciplinary Amyloidosis Network, University Hospital Essen, Essen, Germany.
  • Schönland S; Department of Neurology, University Hospital Essen, Essen, Germany.
  • Rassaf T; Interdisciplinary Amyloidosis Network, University Hospital Essen, Essen, Germany.
  • Reinhardt HC; Department of Nuclear Medicine, University Hospital Essen, Essen, Germany.
  • Jöckel KH; Interdisciplinary Amyloidosis Network, University Hospital Essen, Essen, Germany.
  • Dürig J; Department of Nuclear Medicine, University Hospital Essen, Essen, Germany.
  • Dührsen U; Department of Hematology and Oncology, Oldenburg Hospital, University Medicine Oldenburg, Oldenburg, Germany.
  • Carpinteiro A; Department of Internal Medicine V, Amyloidosis Center Heidelberg, University Hospital Heidelberg, Heidelberg, Germany.
Eur J Haematol ; 107(4): 449-457, 2021 Oct.
Article en En | MEDLINE | ID: mdl-34185342
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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Protocolos de Quimioterapia Combinada Antineoplásica / Diagnóstico Tardío / Tiempo de Tratamiento / Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas / Insuficiencia Cardíaca Tipo de estudio: Diagnostic_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Idioma: En Revista: Eur J Haematol Asunto de la revista: HEMATOLOGIA Año: 2021 Tipo del documento: Article País de afiliación: Alemania Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Protocolos de Quimioterapia Combinada Antineoplásica / Diagnóstico Tardío / Tiempo de Tratamiento / Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas / Insuficiencia Cardíaca Tipo de estudio: Diagnostic_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Idioma: En Revista: Eur J Haematol Asunto de la revista: HEMATOLOGIA Año: 2021 Tipo del documento: Article País de afiliación: Alemania Pais de publicación: Reino Unido