TY  - CHAP
M1  - Book, Section
TI  - Macroglobulinemia
A1  - Treon, Steven P.
A1  - Castillo, Jorge J.
A1  - Hunter, Zachary R.
A1  - Merlini, Giampaolo
A2  - Kaushansky, Kenneth
A2  - Lichtman, Marshall A.
A2  - Prchal, Josef T.
A2  - Levi, Marcel M.
A2  - Press, Oliver W.
A2  - Burns, Linda J.
A2  - Caligiuri, Michael
PY  - 2015
T2  - Williams Hematology, 9e
AB  - SUMMARYWaldenström  macroglobulinemia  (WM)  is  an  indolent  B-cell  neoplasm  manifested  by  the  accumulation  of  clonal  immunoglobulin  (Ig)  M  secreting  lymphoplasmacytic  cells.  MYD88L265P  and  CXCR4  WHIM  (warts,  hypogammaglobulinemia,  infections,  myelokathexis)-like  somatic  mutations  are  present  in  more  than  90  percent,  and  30  to  35  percent  of  WM  patients,  respectively,  and  impact  disease  presentation,  treatment  outcome,  and/or  overall  survival.  Familial  predisposition  is  common  in  WM.  Asymptomatic  patients  should  be  observed.  Patients  with  disease-related  hemoglobin  of  less  than  10g/dL,  platelets  less  than  100  ×  109/L,  bulky  adenopathy  and/or  organomegaly,  symptomatic  hyperviscosity,  peripheral  neuropathy,  amyloidosis,  cryoglobulinemia,  cold-agglutinin  disease,  or  transformed  disease  should  be  considered  for  therapy.  Plasmapheresis  should  be  used  for  patients  with  symptomatic  hyperviscosity,  and  prerituximab  for  those  with  high  serum  IgM  levels  to  preempt  a  symptomatic  IgM  flare.  The  treatment  choice  should  take  into  account  specific  goals  of  therapy,  necessity  for  rapid  disease  control,  risk  of  treatment-related  neuropathy,  immunosuppression  and  secondary  malignancies,  and  planning  for  future  autologous  stem  cell  transplantation.  Frontline  treatments  include  rituximab  alone  or  combined  with  alkylating  agents  (bendamustine,  cyclophosphamide),  proteasome  inhibitors  (bortezomib,  carfilzomib),  or  nucleoside  analogues  (fludarabine,  cladribine).  In  case  of  relapsed  or  treatment-resistant  patients,  an  alternative  frontline  regimen  or  autologous  stem  cell  transplantation  can  be  considered.  Novel  targeted  agents  for  the  treatment  of  WM  include  everolimus  and  ibrutinib.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/19
UR  - hemonc.mhmedical.com/content.aspx?aid=1121101525
ER  -