title: Immunoglobulin light chain amyloidosis is diagnosed late in patients with preexisting plasma cell dyscrasias.
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.
authors.affiliation: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Taxiarchis V
authors.initials: TV
authors.lastname: Kourelis
authors.forename: Shaji K
authors.initials: SK
authors.lastname: Kumar
authors.forename: Ronald S
authors.initials: RS
authors.lastname: Go
authors.forename: Prashant
authors.initials: P
authors.lastname: Kapoor
authors.forename: Robert A
authors.initials: RA
authors.lastname: Kyle
authors.forename: Francis K
authors.initials: FK
authors.lastname: Buadi
authors.forename: Morie A
authors.initials: MA
authors.lastname: Gertz
authors.forename: Martha Q
authors.initials: MQ
authors.lastname: Lacy
authors.forename: Suzanne R
authors.initials: SR
authors.lastname: Hayman
authors.forename: Nelson
authors.initials: N
authors.lastname: Leung
authors.forename: David
authors.initials: D
authors.lastname: Dingli
authors.forename: John A
authors.initials: JA
authors.lastname: Lust
authors.forename: Yi
authors.initials: Y
authors.lastname: Lin
authors.forename: Stephen R
authors.initials: SR
authors.lastname: Zeldenrust
authors.forename: S Vincent
authors.initials: SV
authors.lastname: Rajkumar
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
mesh_terms: D000682:Amyloid / Q000032:analysis*; D000686:Amyloidosis / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D001706:Biopsy; D009202:Cardiomyopathies / Q000175:diagnosis / Q000209:etiology / Q000473:pathology; D016208:Databases, Factual; D057210:Delayed Diagnosis; D003967:Diarrhea / Q000209:etiology; D004066:Digestive System Diseases / Q000175:diagnosis / Q000209:etiology / Q000473:pathology; D004417:Dyspnea / Q000209:etiology; D042241:Early Diagnosis; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis*; D053208:Kaplan-Meier Estimate; D007674:Kidney Diseases / Q000175:diagnosis / Q000209:etiology / Q000473:pathology; D008171:Lung Diseases / Q000175:diagnosis / Q000209:etiology / Q000473:pathology; D010265:Paraproteinemias / Q000150:complications* / Q000401:mortality; D011507:Proteinuria / Q000209:etiology; D012189:Retrospective Studies; D012678:Sensation Disorders / Q000209:etiology; D063189:Symptom Assessment
chemical_list: D000682:Amyloid; D007147:Immunoglobulin Light Chains