A practical approach to the diagnosis of systemic amyloidoses.

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A practical approach to the diagnosis of systemic amyloidoses.

Blood. 2015 Jan 30;

Authors: Fernández de Larrea C, Verga L, Morbini P, Klersy C, Lavatelli F, Foli A, Obici L, Milani P, Capello GL, Paulli M, Palladini G, Merlini G

Accurate diagnosis of systemic amyloidosis is necessary for both assessing the prognosis and to delineate the appropriate treatment. It is based on histological evidence of amyloid deposits and characterization of the amyloidogenic protein. We prospectively evaluated the diagnostic performance of immuno-electron microscopy (IEM) of abdominal fat aspirates from 745 consecutive patients with suspected systemic amyloidoses. All cases were extensively investigated with clinical and laboratory data, with a follow-up of at least 18 months. The 423 (56.8%) cases with confirmed systemic forms were used to estimate the diagnostic performance of IEM. Compared to Congo-red based light microscopy, IEM was equally sensitive (75-80%), whereas IEM revealed significantly more specific (100% vs. 80%; p<0.001). In AL amyloidosis, kappa cases were more difficult to diagnose (sensitivity 71%), while the analysis of abdominal aspirate was informative in only 40% of patients with transthyretin amyloidosis. We found a high prevalence (20%) of a monoclonal component in patients with non-AL amyloidosis highlighting the risk of misdiagnosis and the need for unequivocal amyloid typing. Notably, IEM identified correctly the specific form of amyloidosis in more than 99% of the cases. Immuno-electron microscopy on abdominal fat aspiration is an effective tool in the routine diagnostic of systemic amyloidoses.

PMID: 25636337 [PubMed - as supplied by publisher]

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