• Taxiarchis V Kourelis, Shaji K Kumar, Ronald S Go, Prashant Kapoor, Robert A Kyle, Francis K Buadi, Morie A Gertz, Martha Q Lacy, Suzanne R Hayman, Nelson Leung, David Dingli, John A Lust, Yi Lin, Stephen R Zeldenrust, S Vincent Rajkumar, and Angela Dispenzieri.
• Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
• Am. J. Hematol. 2014 Nov 1; 89 (11): 1051-4.

AbstractAL amyloidosis (AL) is rare and frequently remains undiagnosed until organ function is compromised, even among patients with known pre-existing untreated plasma cell dyscrasias (PCD). We identified 168 patients with AL amyloidosis who had a prior untreated PCD. The earliest symptom or sign (s/s) was defined as the first symptom reported by the patient that could be attributed to organ dysfunction caused by AL. The interval from the time of development of s/s to the establishment of diagnosis of AL (Interval-SA) was calculated. PCD diagnosis preceded recorded onset of s/s in 75% (114/152) of patients, with a median interval-SA for this group of 10 months. PCD was diagnosed after s/s in 25% (38/152) of patients, with a median interval-SA of 20 months. Overall survival (OS) from diagnosis of AL was not different between the two groups. AL amyloidosis patients with an identified pre-existing PCD had less advanced cardiac disease at AL diagnosis when compared to a control group of AL patients without pre-identified PCD. Long-term OS was not significantly superior among patients with a pre-identified PCD. In patients with "asymptomatic" PCD, symptoms and signs of AL amyloidosis should be solicited, since timely diagnosis is important in AL amyloidosis.© 2014 Wiley Periodicals, Inc.

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