A Rare Case of Systemic AL Amyloidosis with Muscle Involvement: A Misleading Diagnosis
Joint Authors
Accardi, Fabrizio
Dalla Palma, Benedetta
Papa, Valentina
Capozzi, Anna Rita
Capello, Gian Luca
Verga, Laura
Mancini, Cristina
Martella, Eugenia
Costa, Roberta
Notarfranchi, Laura
Pietrini, Vladimiro
Cenacchi, Giovanna
Aversa, Franco
Giuliani, Nicola
Source
Issue
Vol. 2018, Issue 2018 (31 Dec. 2018), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2018-01-31
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
Muscle involvement in AL amyloidosis is a rare condition, and the diagnosis of amyloid myopathy is often delayed and underdiagnosed.
Amyloid myopathy may be the initial manifestation and may precede the diagnosis of systemic AL amyloidosis.
Here, we report the case of a 73-year-old man who was referred to our center for a monoclonal gammopathy of undetermined significance (MGUS) diagnosed since 1999.
He reported a progressive weakness of proximal muscles of the legs with onset six months previously.
Muscle biopsy showed mild histopathology featuring alterations of nonspecific type with a mixed myopathic and neurogenic involvement, and the diagnostic turning point was the demonstration of characteristic green birefringence under cross-polarized light following Congo red staining of perimysial vessels.
Transmission electron microscopy (TEM) confirmed amyloid fibrils around perimysial vessels associated with collagen fibrils.
A stepwise approach to diagnosis and staging of this disorder is critical and involves confirmation of amyloid deposition, identification of the fibril type, assessment of underlying amyloidogenic disorder, and evaluation of the extent and severity of amyloidotic organ involvement.
American Psychological Association (APA)
Accardi, Fabrizio& Papa, Valentina& Capozzi, Anna Rita& Capello, Gian Luca& Verga, Laura& Mancini, Cristina…[et al.]. 2018. A Rare Case of Systemic AL Amyloidosis with Muscle Involvement: A Misleading Diagnosis. Case Reports in Hematology،Vol. 2018, no. 2018, pp.1-5.
https://search.emarefa.net/detail/BIM-1144155
Modern Language Association (MLA)
Accardi, Fabrizio…[et al.]. A Rare Case of Systemic AL Amyloidosis with Muscle Involvement: A Misleading Diagnosis. Case Reports in Hematology No. 2018 (2018), pp.1-5.
https://search.emarefa.net/detail/BIM-1144155
American Medical Association (AMA)
Accardi, Fabrizio& Papa, Valentina& Capozzi, Anna Rita& Capello, Gian Luca& Verga, Laura& Mancini, Cristina…[et al.]. A Rare Case of Systemic AL Amyloidosis with Muscle Involvement: A Misleading Diagnosis. Case Reports in Hematology. 2018. Vol. 2018, no. 2018, pp.1-5.
https://search.emarefa.net/detail/BIM-1144155
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1144155