RT Book, Section
A1 Zinzani, Pier Luigi
A1 Broccoli, Alessandro
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
SR Print(0)
ID 1121100619
T1 Marginal Zone B-Cell Lymphomas
T2 Williams Hematology, 9e
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 9780071833004
LK accessmedicine.mhmedical.com/content.aspx?aid=1121100619
RD 2024/04/18
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  are  overlapping.  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  (e.g.,  Helicobacter  pylori  in  the  stomach)  or  an  autoimmune  disease  (as  Sjögren  syndrome  or  Hashimoto  thyroiditis).  SMZL  accounts  for  approximately  20  percent  of  all  marginal  zone  lymphomas,  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  percent  of  all  marginal  zone  lymphomas  and  typically  presenting  with  lymph  node-based  disease  without  splenic  or  extranodal  site  involvement.