title: Management of systemic AL amyloidosis: recommendations of the Myeloma Foundation of Australia Medical and Scientific Advisory Group.
abstract: Systemic AL amyloidosis is a plasma cell dyscrasia with a characteristic clinical phenotype caused by multi-organ deposition of an amyloidogenic monoclonal protein. This condition poses a unique management challenge due to the complexity of the clinical presentation and the narrow therapeutic window of available therapies. Improved appreciation of the need for risk stratification, standardised use of sensitive laboratory testing for monitoring disease response, vigilant supportive care and the availability of newer agents with more favourable toxicity profiles have contributed to the improvement in treatment-related mortality and overall survival seen over the past decade. Nonetheless, with respect to the optimal management approach, there is a paucity of high-level clinical evidence due to the rarity of the disease, and enrollment in clinical trials is still the preferred approach where available. This review will summarise the Clinical Practice Guidelines on the Management of Systemic Light Chain (AL) Amyloidosis recently prepared by the Medical Scientific Advisory Group of the Myeloma Foundation of Australia. It is hoped that these guidelines will assist clinicians in better understanding and optimising the management of this difficult disease.
authors.affiliation: Clinical Haematology and Bone Marrow Transplant Unit, Royal Brisbane and Women's Hospital, Brisbane, Australia.
authors.forename: N
authors.initials: N
authors.lastname: Weber
authors.forename: P
authors.initials: P
authors.lastname: Mollee
authors.forename: B
authors.initials: B
authors.lastname: Augustson
authors.forename: R
authors.initials: R
authors.lastname: Brown
authors.forename: L
authors.initials: L
authors.lastname: Catley
authors.forename: J
authors.initials: J
authors.lastname: Gibson
authors.forename: S
authors.initials: S
authors.lastname: Harrison
authors.forename: P J
authors.initials: PJ
authors.lastname: Ho
authors.forename: N
authors.initials: N
authors.lastname: Horvath
authors.forename: W
authors.initials: W
authors.lastname: Jaksic
authors.forename: D
authors.initials: D
authors.lastname: Joshua
authors.forename: H
authors.initials: H
authors.lastname: Quach
authors.forename: A W
authors.initials: AW
authors.lastname: Roberts
authors.forename: A
authors.initials: A
authors.lastname: Spencer
authors.forename: J
authors.initials: J
authors.lastname: Szer
authors.forename: D
authors.initials: D
authors.lastname: Talaulikar
authors.forename: B
authors.initials: B
authors.lastname: To
authors.forename: A
authors.initials: A
authors.lastname: Zannettino
authors.forename: H M
authors.initials: HM
authors.lastname: Prince
mesh_terms: D026683:Advisory Committees / Q000592:standards*; D000686:Amyloidosis / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D001315:Australia / Q000453:epidemiology; D019468:Disease Management; D005582:Foundations / Q000592:standards*; D006801:Humans; D009101:Multiple Myeloma / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*
keywords: AL; amyloidosis; light chain; plasma cell