TY  - CHAP
M1  - Book, Section
TI  - Macroglobulinemia
A1  - Treon, Steven P.
A1  - Castillo, Jorge J.
A1  - Hunter, Zachary R.
A1  - Merlini, Giampaolo
A2  - Press, Oliver W.
A2  - Lichtman, Marshall A.
A2  - Leonard, John P.
PY  - 2017
T2  - Williams Hematology Malignant Lymphoid Diseases
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  10  g/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);  nucleoside  analogues  (fludarabine,  cladribine);  and  ibrutinib.  In  case  of  relapsed  or  treatment-resistant  patients,  an  alternative  frontline  regimen  or  autologous  stem  cell  transplantation  can  be  considered.  Everolimus  can  be  considered  for  those  patients  with  multiple  relapses.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/19
UR  - hemonc.mhmedical.com/content.aspx?aid=1148369807
ER  -