RT Book, Section
A1 Broccoli, Alessandro
A1 Zinzani, Pier Luigi
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 1180444347
T1 Marginal Zone B-Cell Lymphomas
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=1180444347
RD 2024/04/20
AB SUMMARYIndolent  B-cell  lymphomas  deriving  from  the  marginal  zone  include  three  specific  entities:  extranodal  marginal  zone  (or  mucosa-associated  lymphoid  tissue)  lymphoma  (EMZL),  splenic  marginal  zone  lymphoma  (SMZL),  and  nodal  marginal  zone  lymphoma  (NMZL).  The  clinical  and  molecular  characteristics  are  distinctive  for  each  of  these  entities,  although  some  phenotypic  and  genetic  features  overlap.  EMZL  is  the  most  common  entity,  arising  at  virtually  any  extranodal  site,  commonly  associated  with  chronic  antigenic  stimulation  either  as  a  result  of  an  external  infection  (eg,  Helicobacter  pylori  in  the  stomach)  or  an  autoimmune  disease  (eg,  Sjögren  syndrome  or  Hashimoto  thyroiditis).  SMZL  accounts  for  approximately  20%  of  all  marginal  zone  lymphomas  (MZLs),  with  patients  typically  presenting  with  an  enlarged  spleen  and  involvement  of  marrow  and  splenic  hilar  lymph  nodes.  NMZL  is  the  least  common  entity,  representing  approximately  10%  of  all  MZLs  and  typically  presenting  with  lymph  node–based  disease  without  splenic  or  extranodal  site  involvement.