TY  - CHAP
M1  - Book, Section
TI  - Plasma Cell Disorders
A1  - Rowe, Julie H.
A1  - Gonzalez, Anneliese O.
A1  - Jafri, Syed H.
A1  - Cen, Putao
A1  - Kanaan, Zeyad
A1  - Amato, Robert J.
A1  - Rios, Adan
A1  - El-Osta, Hazem
A1  - Mohlere, Virginia
Y1  - 2019
N1  - 
T2  - Hematology-Oncology Clinical Questions
AB  - Table  Graphic  Jump  Location|Download  (.pdf)|PrintKey  conceptThe  cardinal  feature  of  plasma  cell  disorders  is  the  abnormal  increased  production  of  monoclonal  antibodies.  Thus,  abnormal  elevation  of  serum  proteins  should  always  raise  suspicion  of  a  plasma  cell  neoplasia.Clinical  scenarioA  65-year-old  woman  presents  with  a  history  of  anemia  of  recent  onset.  A  complete  physical  examination  is  unrevealing.  Complete  blood  chemistry  shows  an  elevated  total  protein.  Serum  albumin  is  normal.  Serum  electrophoresis  is  ordered.Action  itemOrder  a  serum  electrophoresis,  a  serum  immune-electrophoresis,  and  a  urine  spot  for  Bence-Jones  proteinDiscussionMultiple  myeloma  (MM)  is  a  malignant  neoplasm  of  plasma  cells  that  accounts  for  1.8%  of  all  cancers  and  ~17%  of  all  hematological  malignancies  in  the  United  States.  It  is  most  frequently  diagnosed  among  people  aged  65–74  years,  with  the  median  age  being  69  years.  The  American  Cancer  Society  has  estimated  close  to  30,000  cases  of  myeloma  newly  diagnosed  in  2017.  The  approximate  death  rate  is  12,000  deaths  per  year.1  For  a  patient  to  be  diagnosed  with  MM,  they  must  have  not  only  a  plasma  cell  disorder  but  also  end-organ  damage  attributable  to  the  plasma  cell  disorder.  This  includes  at  least  one  of  the  CRAB  criteria  (hyper-Calcemia,  Renal  disease,  Anemia,  and  Bone  disease)  classically  used  to  define  symptomatic  MM.  Anemia  occurs  in  73%  of  patients.  Bone  pain  is  common  (60%  of  patients),  and  50%  of  patients  have  an  elevated  serum  creatinine.  Renal  disease  results  from  light  precipitated  complexes  obstructing  the  distal  convoluted  tubules.  Other  causes  of  renal  disease  include  nephrocalcinosis,  amyloidosis,  heavy-chain  disease,  and  light-chain  disease.  Hypercalcemia  >11  mg/dL  is  present  in  10%  of  patients.  This  requires  hydration  with  isotonic  saline  and  bisphosphonate  therapy  with  zoledronic  acid  or  pamidronate  in  moderate  or  severe  cases.  Calcitonin  can  also  be  used  to  rapidly  reduce  serum  calcium  levels.  Other  symptoms  include  fatigue  (32%)  and  weight  loss  (20%).  Because  of  immune  dysfunction,  patients  are  at  risk  for  infections.  About  7%–18%  of  patients  may  present  with  extramedullary  plasmacytomas.  Less  common  symptoms  include  fever,  splenomegaly,  hepatomegaly,  and  lymphadenopathy.PearlIf  there  is  elevated  serum  protein,  the  critical  differentiation  to  be  made  is  whether  the  elevation  is  associated  with  a  monoclonal  gammopathy  or  is  a  nonspecific  elevation  of  the  gamma  region  associated  with  a  chronic  inflammatory  processReferencesKyle  Ra,  Gertz  MA,  Witzig  TE,  et  al.  Review  of  1027  patients  with  newly  diagnosed  multiple  myeloma.  Mayo  Clin  Proc  2003;78:21-33.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - hemonc.mhmedical.com/content.aspx?aid=1162981498
ER  -