Immunoglobulin light chain amyloidosis (AL)

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Light chain amyloidosis needs to be considered in the differential diagnosis of patients with nondiabetic nephrotic range proteinuria, unexplained fatigue or heart failure with preserved ejection fraction, a progressive peripheral neuropathy associated with a monoclonal protein, or atypical multiple myeloma. Screening involves serum and urine immunofixation and an immunoglobulin free light chain assay. Most patients can have the diagnosis established via biopsy of the bone marrow and subcutaneous fat. Visceral organ biopsy is rarely needed. The prognosis is determined by the DFLC, the levels of cardiac biomarkers, troponin and BNP. Therapy includes high-dose chemotherapy; and for patients ineligible for high-dose chemotherapy, bortezomib appears to be the single most active agent. Combinations of bortezomib and the inclusion of monoclonal antibodies are the subject of further studies.

Original languageEnglish (US)
Title of host publicationNeoplastic Diseases of the Blood
PublisherSpringer International Publishing
Pages651-672
Number of pages22
ISBN (Electronic)9783319642635
ISBN (Print)9783319642628
DOIs
StatePublished - Jan 1 2018

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Keywords

  • Amyloidosis
  • Bortezomib
  • Cardiac biomarkers
  • Congo red
  • Fat aspiration
  • Heart failure
  • Immunoglobulin light chain
  • Lenalidomide
  • Melphalan
  • Multiple myeloma
  • Nephrotic syndrome
  • Peripheral neuropathy
  • Stem cell transplant
  • Thalidomide

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Gertz, M., Buadi, F. K., Sher, T., & Dispenzieri, A. (2018). Immunoglobulin light chain amyloidosis (AL). In Neoplastic Diseases of the Blood (pp. 651-672). Springer International Publishing. https://doi.org/10.1007/978-3-319-64263-5_36