RT Book, Section
A1 Lichtman, Marshall A.
A2 Kaushansky, Kenneth
A2 Prchal, Josef T.
A2 Burns, Linda J.
A2 Lichtman, Marshall A.
A2 Levi, Marcel
A2 Linch, David C.
SR Print(0)
ID 1178752222
T1 Essential Monoclonal Gammopathy
T2 Williams Hematology, 10e
YR 2021
FD 2021
PB McGraw-Hill Education
PP New York, NY
SN 9781260464122
LK hemonc.mhmedical.com/content.aspx?aid=1178752222
RD 2024/04/19
AB SUMMARYEssential  monoclonal  gammopathy  is  defined  by  two  key  features:  (a)  the  presence  of  a  monoclonal  immunoglobulin  (Ig)  or  a  monoclonal  Ig  light  chain  in  the  serum  and  (b)  the  absence  of  evidence  for  an  overt  malignancy  of  B  lymphocytes  or  plasma  cells  (eg,  lymphoma,  myeloma,  or  amyloidosis).  The  prevalence  of  essential  monoclonal  gammopathy  depends  on  the  demographic  features  in  the  population  under  study.  In  Americans  of  European  descent,  the  prevalence  increases  from  approximately  2%  in  individuals  50  years  of  age  to  approximately  7%  in  octogenarians.  It  is  two  to  three  times  as  prevalent  in  persons  of  African  descent.  The  condition  has  been  reported  in  association  with  a  large  variety  of  disorders,  especially  nonlymphocytic  cancers.  These  coincidences  are  thought,  in  most  cases,  to  be  the  chance  concurrence  of  conditions  that  have  a  high  prevalence  in  older  persons.  Some  cases  of  essential  monoclonal  gammopathy  are  symptomatic  because  in  these  cases,  the  Ig  can  interact  with  plasma  proteins,  blood  cells,  kidney,  ocular  structures,  or  neural  tissue  and  cause  serious  dysfunction,  for  example,  an  acquired  bleeding  disorder,  renal  insufficiency,  or  an  incapacitating  neuropathy.  In  such  cases,  disability  may  be  so  great  that  attempts  to  remove  the  Ig  by  plasmapheresis  and  to  suppress  its  production  using  immune  or  cytotoxic  therapy  can  be  warranted.  Because  myeloma  or  lymphoma  may  emerge  soon  after  the  monoclonal  Ig  is  first  detected,  subsequent  evaluation  of  the  patient  is  required  to  ascertain  if  essential  monoclonal  gammopathy  is  the  appropriate  diagnosis.  Long-term  follow-up  at  appropriate  intervals  is  prudent  to  detect  conversion  from  a  stable,  asymptomatic  condition  to  a  progressive  lymphoma  or  myeloma,  which  occurs  in  approximately  0.75%  of  cases  per  year.  In  the  absence  of  a  symptomatic  monoclonal  gammopathy  (eg,  renal  or  neurologic  involvement)  or  evolution  to  a  progressive  clonal  gammopathy,  periodic  follow-up  of  patients  is  all  that  is  required.