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Blood, 1 May 2006, Vol. 107, No. 9, pp. 3489-3491.
Prepublished online as a Blood First Edition Paper on January 26, 2006; DOI 10.1182/blood-2005-10-4148.
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Submitted October 19, 2005
Accepted December 14, 2005
Seeking confidence in the diagnosis of systemic AL (Ig light-chain) amyloidosis: patients can have both monoclonal gammopathies and hereditary amyloid proteins
Raymond L Comenzo*, Ping Zhou, Martin Fleisher, Bradly Clark, and Julie Teruya-Feldstein
Hematology Service, Sloan-Kettering Institute, New York, New York, USA; Department of Clinical Laboratories, Memorial Sloan-Kettering Cancer Center, New York, New York, USA
Division of Hematologic Oncology, Department of Medicine, Sloan-Kettering Institute, New York, New York, USA
Department of Clinical Laboratories, Memorial Sloan-Kettering Cancer Center, New York, New York, USA
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York, USA
* Corresponding author; email: comenzor{at}mskcc.org.
Investigators in the United Kingdom have shown that hereditary amyloidosis can be misdiagnosed as Ig light-chain (AL) amyloidosis because family history is an ineffective screen and tissue-staining used to type amyloid is unreliable. Misdiagnosis of AL can lead to inappropriate use of chemotherapy and failure to diagnose a hereditary disease. Over a 3-year period we sought to determine how often both possible sources of amyloidosis occurred in the same patient. We employed an algorithm based on established data and patterns of amyloidosis in order to focus the screening effort. Of 178 consecutive patients referred for amyloidosis, 54 were screened by polymerase chain reaction techniques with primers designed to detect transthyretin, apolipoprotein AI, apolipoprotein AII, fibrinogen A and lysozyme variants. Three patients (6% of those screened and 2% of symptomatic patients) had both a monoclonal gammopathy and a hereditary variant. These results justify further study of screening for hereditary variants in patients with apparent AL, and highlight the need for practical techniques for identifying fibrils extracted from tissue.
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