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

Am J Hematol. 2014 Nov;89(11):1051-4. doi: 10.1002/ajh.23827. Epub 2014 Sep 2.

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.

MeSH terms

  • Amyloid / analysis*
  • Amyloidosis / diagnosis*
  • Amyloidosis / etiology
  • Amyloidosis / pathology
  • Biopsy
  • Cardiomyopathies / diagnosis
  • Cardiomyopathies / etiology
  • Cardiomyopathies / pathology
  • Databases, Factual
  • Delayed Diagnosis
  • Diarrhea / etiology
  • Digestive System Diseases / diagnosis
  • Digestive System Diseases / etiology
  • Digestive System Diseases / pathology
  • Dyspnea / etiology
  • Early Diagnosis
  • Humans
  • Immunoglobulin Light Chains / analysis*
  • Kaplan-Meier Estimate
  • Kidney Diseases / diagnosis
  • Kidney Diseases / etiology
  • Kidney Diseases / pathology
  • Lung Diseases / diagnosis
  • Lung Diseases / etiology
  • Lung Diseases / pathology
  • Paraproteinemias / complications*
  • Paraproteinemias / mortality
  • Proteinuria / etiology
  • Retrospective Studies
  • Sensation Disorders / etiology
  • Symptom Assessment

Substances

  • Amyloid
  • Immunoglobulin Light Chains