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: Article (Published version)

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.)