RT Book, Section
A1 Kaufman, Gregory P.
A1 Qazilbash, Muzaffar H.
A1 Patel, Krina
A1 Thomas, Sheeba
A1 Orlowski, Robert Z.
A1 Lee, Hans C.
A2 Kantarjian, Hagop M.
A2 Wolff, Robert A.
A2 Rieber, Alyssa G.
SR Print(0)
ID 1190833527
T1 Systemic Immunoglobulin Light Chain Amyloidosis
T2 The MD Anderson Manual of Medical Oncology, 4e
YR 2022
FD 2022
PB McGraw Hill Education
PP New York, NY
SN 9781260467642
LK hemonc.mhmedical.com/content.aspx?aid=1190833527
RD 2024/04/25
AB KEY  CONCEPTSSystemic  light  chain  (AL)  amyloidosis  is  a  rare  protein  deposition  disease  typically  found  accompanied  by  an  indolent  plasma  cell  dyscrasia,  although  it  can  also  be  found  concomitantly  with  multiple  myeloma.In  the  systemic  presentation  (some  cases  are  localized),  commonly  affected  organs  may  include  the  heart,  kidneys,  liver,  gastrointestinal  tract,  and  peripheral  nervous  system.Diagnosis  requires  histologic  evidence  of  amyloid  deposition  in  tissues  either  by  aspiration  of  abdominal  subcutaneous  fat  or  biopsy  of  the  organs  involved,  with  the  demonstration  of  clonal  plasma  cell  disorder  and  abnormal  free  light  chain  in  serum  or  urine.Historical  cohorts  of  patients  with  severe  cardiac  involvement  have  a  poor  prognosis  of  4  to  6  months,  though  recent  studies  show  this  can  be  greatly  improved  with  successful  treatment.Plasma  cell–directed  therapy  with  or  without  high-dose  melphalan  and  autologous  stem  cell  rescue  is  the  treatment  of  choice  for  patients  with  confirmed  systemic  AL.Multidisciplinary  care  is  beneficial  because  these  patients  have  distinct  clinical  presentations,  symptoms,  and  risks  compared  with  patients  with  other  plasma  cell  dyscrasias.