RT Book, Section
A1 Bianchi, Giada
A1 O’Donnell, Elizabeth K.
A1 Anderson, Kenneth
A2 Kaushansky, Kenneth
A2 Prchal, Josef T.
A2 Burns, Linda J.
A2 Lichtman, Marshall A.
A2 Levi, Marcel
A2 Linch, David C.
SR Print(0)
ID 1178752132
T1 Plasma Cell Neoplasms: General Considerations
T2 Williams Hematology, 10e
YR 2021
FD 2021
PB McGraw-Hill Education
PP New York, NY
SN 9781260464122
LK hemonc.mhmedical.com/content.aspx?aid=1178752132
RD 2025/07/20
AB SUMMARYPlasma  cell  neoplasms  (PCNs)  are  derived  from  the  expansion  of  mutated,  terminally  differentiated,  postgerminal  center  B  cells.  These  neoplasms  include  monoclonal  gammopathy  of  undetermined  significance  (MGUS)  and  monoclonal  gammopathy  of  renal  significance  (MGRS)  (Chap.  105),  smoldering  myeloma  (Chap.  106),  myeloma  (Chap.  106),  solitary  plasmacytomas  and  plasmacytomas  with  minimal  marrow  involvement  (Chap.  106),  and  light-chain  amyloidosis  (Chap.  107).  The  prototypic  malignant  PCN  is  myeloma,  which  is  characterized  by  complex  genetic  alterations,  best  assessed  by  metaphase  cytogenetics,  fluorescence  in  situ  hybridization  analysis,  and  gene  expression  profiling.  The  genetic  changes  are  more  akin  to  solid  tumors  than  to  hematologic  malignancies.  Interactions  between  myeloma  cells  and  the  marrow  microenvironment  affect  the  survival,  proliferation,  and  drug  resistance  of  myeloma  cells  and  the  development  of  osteoporosis  or  osteolysis,  which  is  a  hallmark  of  myeloma.  As  in  most  malignancies,  a  putative  cancer  stem  cell  (eg,  myeloma  stem  cell)  has  been  identified  and  is  the  most  likely  site  of  drug  resistance,  which  almost  invariably  develops  during  treatment;  such  cells  are  not  affected  by  the  typical  drugs  used  in  patients  with  myeloma.  Patients  can  be  prognostically  stratified  based  on  the  International  Staging  System  (ISS)  and  revised  ISS,  which  includes  cytogenetics  and  lactate  dehydrogenase  as  well  as  albumin  and  β2  microglobulin.  The  use  of  combination  chemotherapy  regimens  incorporating  drugs  targeting  myeloma  in  the  context  of  the  marrow  microenvironment,  such  as  proteasome  inhibitors,  immunomodulatory  drugs,  and  monoclonal  antibodies,  alone  or  in  combination  with  transplantation,  has  significantly  improved  depth  and  duration  of  response,  resulting  in  increased  survival.  Minimal  residual  disease  negative  status  is  a  strong  prognostic  factor  for  progression-free  and  overall  survival  and  is  becoming  an  important  endpoint  in  clinical  trials,  for  new  drug  registration,  and  to  inform  clinical  practice.