Coexistence of Positive 99mTc-DPD Scintigraphy and Monoclonal Gammopathy: A Frequent Challenge
Roteta Unceta-Barrenechea, Ana ; Melero Polo, Jorge ; Andrés Gracia, Alejandro (Universidad de Zaragoza) ; Revilla Martí, Pablo ; Menao Guillén, Sebastian ; Lahuerta Pueyo, Carmen ; Pérez-Palacios, Raquel (Universidad de Zaragoza) ; Moreno Gázquez, Inmaculada ; Gracia Gutiérrez, Anyuli ; Aibar Arregui, Miguel Angel
Resumen: Background
Cardiac involvement is common in amyloidosis, and the vast majority of cases of amyloid cardiomyopathy are attributed to primary amyloidosis or transthyretin amyloidosis (ATTR). Although the coexistence of scintigraphy suggestive of ATTR with monoclonal gammopathy of undetermined significance is well documented, the correct diagnosis is still challenging in non-referral centers.
Methods
We performed a retrospective study reviewing all amyloid cardiomyopathy cases diagnosed at our center over the last 5 years, and described our experience and diagnostic approach.
Results
During the last 5 years, 74 patients with positive scintigraphy were identified. Of these patients, 41 were included in this study as they had all necessary tests for a complete diagnosis. Two of these 41 patients had variant ATTR and 29 had wild-type ATTR. Ten patients had monoclonal gammopathy (24.4%), and it was consequently impossible to obtain a specific diagnosis. During follow-up, 14 patients (34.1%), five of them from the monoclonal gammopathy group, died, reflecting the severity of disease.
Conclusions
In patients with ATTR-suggestive scintigraphy, monoclonal gammopathy frequently occurs concomitantly, thus not allowing to establish a specific diagnosis. A biopsy could only be replaced by genetic testing in selected cases.
Idioma: Inglés
DOI: 10.6515/ACS.202203_38(2).20210927A
Año: 2022
Publicado en: Acta Cardiologica Sinica 38, 2 (2022), 169-174
ISSN: 1011-6842
Originalmente disponible en: Texto completo de la revista
Factor impacto JCR: 1.9 (2022)
Categ. JCR: CARDIAC & CARDIOVASCULAR SYSTEMS rank: 108 / 143 = 0.755 (2022) - Q4 - T3
Factor impacto CITESCORE: 3.0 - Medicine (Q3)
Factor impacto SCIMAGO: 0.338 - Cardiology and Cardiovascular Medicine (Q3)
Tipo y forma: Artículo (Versión definitiva)
Área (Departamento): Área Radiol. y Medicina Física (Dpto. Microb.Ped.Radio.Sal.Pú.)
Área (Departamento): Área Anatom.Anatom.Patológ.Com (Dpto. Anatom.,Embri.Genét.Ani.)
Derechos reservados por el editor de la revista
Exportado de SIDERAL (2024-03-18-17:08:23)
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Cardiac involvement is common in amyloidosis, and the vast majority of cases of amyloid cardiomyopathy are attributed to primary amyloidosis or transthyretin amyloidosis (ATTR). Although the coexistence of scintigraphy suggestive of ATTR with monoclonal gammopathy of undetermined significance is well documented, the correct diagnosis is still challenging in non-referral centers.
Methods
We performed a retrospective study reviewing all amyloid cardiomyopathy cases diagnosed at our center over the last 5 years, and described our experience and diagnostic approach.
Results
During the last 5 years, 74 patients with positive scintigraphy were identified. Of these patients, 41 were included in this study as they had all necessary tests for a complete diagnosis. Two of these 41 patients had variant ATTR and 29 had wild-type ATTR. Ten patients had monoclonal gammopathy (24.4%), and it was consequently impossible to obtain a specific diagnosis. During follow-up, 14 patients (34.1%), five of them from the monoclonal gammopathy group, died, reflecting the severity of disease.
Conclusions
In patients with ATTR-suggestive scintigraphy, monoclonal gammopathy frequently occurs concomitantly, thus not allowing to establish a specific diagnosis. A biopsy could only be replaced by genetic testing in selected cases.
Idioma: Inglés
DOI: 10.6515/ACS.202203_38(2).20210927A
Año: 2022
Publicado en: Acta Cardiologica Sinica 38, 2 (2022), 169-174
ISSN: 1011-6842
Originalmente disponible en: Texto completo de la revista
Factor impacto JCR: 1.9 (2022)
Categ. JCR: CARDIAC & CARDIOVASCULAR SYSTEMS rank: 108 / 143 = 0.755 (2022) - Q4 - T3
Factor impacto CITESCORE: 3.0 - Medicine (Q3)
Factor impacto SCIMAGO: 0.338 - Cardiology and Cardiovascular Medicine (Q3)
Tipo y forma: Artículo (Versión definitiva)
Área (Departamento): Área Radiol. y Medicina Física (Dpto. Microb.Ped.Radio.Sal.Pú.)
Área (Departamento): Área Anatom.Anatom.Patológ.Com (Dpto. Anatom.,Embri.Genét.Ani.)
Derechos reservados por el editor de la revistaExportado de SIDERAL (2024-03-18-17:08:23)
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Registro creado el 2024-01-30, última modificación el 2024-03-19