A Rare Case of Systemic AL Amyloidosis with Muscle Involvement: A Misleading Diagnosis
| العنوان: | A Rare Case of Systemic AL Amyloidosis with Muscle Involvement: A Misleading Diagnosis |
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| المؤلفون: | Franco Aversa, Valentina Papa, Benedetta Dalla Palma, Nicola Giuliani, Cristina Mancini, Roberta Costa, Anna Rita Capozzi, Laura Verga, V. Pietrini, Laura Notarfranchi, Fabrizio Accardi, Giovanna Cenacchi, Eugenia Martella, Gian Luca Capello |
| المساهمون: | Accardi F, Papa V, Capozzi AR, Capello GL, Verga L, Mancini C, Martella E, Costa R, Notarfranchi L, Dalla Palma B, Aversa, Pietrini V3, Cenacchi G, Giuliani N. |
| المصدر: | Case Reports in Hematology, Vol 2018 (2018) Case Reports in Hematology |
| بيانات النشر: | Hindawi Limited, 2018. |
| سنة النشر: | 2018 |
| مصطلحات موضوعية: | 030213 general clinical medicine, Pathology, medicine.medical_specialty, Weakness, LIGHT-CHAIN AMYLOIDOSIS, Case Report, Fibril, PATIENT, DISEASE, 03 medical and health sciences, MYOPATHY, 0302 clinical medicine, Perimysial, medicine, AL amyloidosis, Myopathy, Muscle biopsy, medicine.diagnostic_test, business.industry, lcsh:RC633-647.5, General Medicine, lcsh:Diseases of the blood and blood-forming organs, medicine.disease, Histopathology, medicine.symptom, business, 030217 neurology & neurosurgery, Monoclonal gammopathy of undetermined significance |
| الوصف: | 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. |
| وصف الملف: | ELETTRONICO |
| اللغة: | English |
| تدمد: | 2090-6579 2090-6560 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::663b54d553a5d0807876f31a1b1fb0b0Test https://doaj.org/article/f5fdd812a8404bcfadcc6292e480c966Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....663b54d553a5d0807876f31a1b1fb0b0 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 20906579 20906560 |
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