Immunoglobulin light chain amyloidosis is diagnosed late in patients with preexisting plasma cell dyscrasias

Taxiarchis Kourelis, Shaji K Kumar, Ronald S. Go, Prashant Kapoor, Robert A. Kyle, Francis K. Buadi, Morie Gertz, Martha Lacy, Suzanne R. Hayman, Nelson Leung, David M Dingli, John A. Lust, Yi Lin, Stephen R. Zeldenrust, S Vincent Rajkumar, Angela Dispenzieri

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Abstract

AL 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.

Original languageEnglish (US)
Pages (from-to)1051-1054
Number of pages4
JournalAmerican Journal of Hematology
Volume89
Issue number11
DOIs
StatePublished - Nov 1 2014

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ASJC Scopus subject areas

  • Hematology

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