RT Book, Section
A1 Tricot, Guido
A1 Janz, Siegfried
A1 Nadiminti, Kalyan
A1 Wendlandt, Erik
A1 Zhan, Fenghuang
A2 Press, Oliver W.
A2 Lichtman, Marshall A.
A2 Leonard, John P.
SR Print(0)
ID 1148369299
T1 Plasma Cell Neoplasms: General Considerations
T2 Williams Hematology Malignant Lymphoid Diseases
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9781260117066
LK hemonc.mhmedical.com/content.aspx?aid=1148369299
RD 2024/04/19
AB SUMMARYPlasma  cell  neoplasms  are  tumors  derived  from  an  expansion  of  mutated  mature  B-cells  and  their  precursors.  These  neoplasms  include  essential  monoclonal  gammopathy  (synonym:  monoclonal  gammopathy  of  unknown  significance;  Chap.  17),  smoldering  myeloma  (Chap.  18),  myeloma  (Chap.  18),  solitary  and  extramedullary  plasmacytomas  (Chap.  18),  light-chain  amyloidosis  (Chap.  19),  and  Waldenström  macroglobulinemia  (Chap.  20).  The  prototype  of  a  malignant  plasma  cell  neoplasm  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  cancer  stem  cell  (e.g.,  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  one  uses  in  patients  with  myeloma.  The  best  prognostic  markers  in  myeloma  in  order  of  importance  are  the  presence  of  (1)  specific  cytogenetic  abnormalities,  (2)  extent  of  the  disease  by  appropriate  imaging  techniques,  such  as  magnetic  resonance  imaging  and/or  combined  positron  emission  and  computed  tomographic  imaging,  (3)  the  serum-free  light-chain  level  and  kappa-to-lambda  ratio,  and  (4)  the  use  of  the  International  Staging  System.  The  development  of  several  classes  of  drugs  over  the  past  decade  in  combination  with  transplantation  has  improved  therapeutic  outcomes  significantly  in  patients  achieving  an  unequivocal  complete  remission.  Thus,  optimal  techniques  to  assess  minimal  residual  disease  have  also  become  important.