RT Book, Section
A1 Becnel, Melody
A1 Kaufman, Gregory P.
A1 Manasanch, Elisabet E.
A1 Patel, Krina
A1 Lee, Hans C.
A1 Orlowski, Robert Z.
A1 Thomas, Sheeba
A2 Kantarjian, Hagop M.
A2 Wolff, Robert A.
A2 Rieber, Alyssa G.
SR Print(0)
ID 1190833558
T1 Waldenström Macroglobulinemia
T2 The MD Anderson Manual of Medical Oncology, 4e
YR 2022
FD 2022
PB McGraw Hill Education
PP New York, NY
SN 9781260467642
LK hemonc.mhmedical.com/content.aspx?aid=1190833558
RD 2024/04/23
AB KEY  CONCEPTSWaldenström  macroglobulinemia  (WM)  is  synonymous  with  lymphoplasmacytic  lymphoma  as  well  as  the  presence  of  a  monoclonal  immunoglobulin  (Ig)  M  gammopathy.Most  cases  are  associated  with  a  mutation  in  MYD88,  which  offers  a  better  prognosis  than  MYD88  wild-type  cases.CXCR4  mutations  are  associated  with  increased  IgM  levels  and  a  higher  risk  of  hyperviscosity.Patients  with  WM  are  at  risk  for  symptomatic  hyperviscosity  syndrome,  which  may  present  with  visual  disturbances,  dizziness,  cardiopulmonary  symptoms,  decreased  consciousness,  and  a  bleeding  diathesis.  Therapy  for  hyperviscosity  consists  of  prompt  initiation  of  plasma  exchange  followed  by  systemic  therapy.As  with  other  low-grade  lymphoid  malignancies,  asymptomatic  patients  and  those  without  significant  cytopenias  or  other  end-organ  manifestations  can  be  observed  with  close  follow-up.Therapy  should  be  initiated  for  symptomatic  hyperviscosity,  hemoglobin  less  than  10  g/dL,  platelet  count  100,000  K/uL  or  less,  bulky  adenopathy,  symptomatic  organomegaly,  symptomatic  cryoglobulinemia,  or  significant  peripheral  neuropathy