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CHAPTER 22  ■  Lymphoid and Plasma Cell Neoplasms                                  433







































                   FIGURE  22.14  Ree  -Sternberg cell typic  l o  Ho  gkin   ise  se.

                   (Reprinte    ro   Rubin E, F  rber JL. Pathology, 3r   e  , Phil    elphi  ,

                   PA: Lippincott Willi    s & Wilkins, 1999, with per  ission.)

                                                                                                                               FIGURE  22.16  Cl  ssic  l  Ho  gkin  ly  pho    .  CD30-positive

                       v  nces,    neutrophili   with tot  l leukocyte counts o  15                                            Ree  -Sternberg  cells.  (Cytoche  ic  l  st  in.)  (Reprinte     ro


                   to  25  ×  10 /L    evelops.  Neutrophili  ,  v  rying    egrees  o                                         Or  zi A, Fouc  r K, Knowles D, Weiss LM. Knowles Neoplastic
                                       9
                   eosinophili  ,   n     onocytosis beco  e   pp  rent on periph-                                             Hematopathology, 3r   e  , Phil    elphi  , PA: Lippincott Willi    s &


                   er  l s  e  rs   s the   ise  se progresses. In the l  ter st  ges o                                        Wilkins, 2013, with per  ission.)
                   the    isor  er,    ost  p  tients    evelop  ly  phocytopeni      n


                   thro  bocytopeni  .                                                                                         IL-2  pro  uction,    n    incre  se    sensitivity  to  suppressor
                        Multinucle  te    gi  nt  cells  or  l  rge    ononucle  r  cell                                        onocytes   n   nor   l   -suppressor cells.


                   v  ri  nts (ly  phocyte   n   histiocyte)   re seen in Ho  gkin                                                  T e cellul  r origin o  the Ree  -Sternberg cell is unknown,
                    ise  se. T ese cells   ccount  or   bout 1% o  the cells in                                                but  Ree  -Sternberg  cells  h  ve  been  shown  to   unction    s


                   b  ckgroun   o  in          tory cells consisting o  s    ll ly  -                                          sti  ul  tory cells in     ny ly  phocyte re  ctions,   s   ccessory
                   phocytes, histiocytes, neutrophils, eosinophils,   n   pl  s                                                cells in   itogen-in  uce     -cell proli er  tion,   n     s   ntigen-


                   cells.                                                                                                      presenting  cells  in  HLA-DR–restricte  ,    ntigen-specif c
                        Ho  gkin   ise  se is ch  r  cterize   by the presence o  Ree  -                                         -cell   ctiv  tion.


                   Sternberg cells (Fig. 22.14) in the ly  ph no  es. T e no  es   n
                   other ly  phoi   tissue   re o  en inf ltr  te   with  ly  phocytes,                                        Prognosis


                   reticulu    cells,  f brocytes,  pl  s      cells,    onocytes,    n                                        Ho  gkin ly  pho     is    high cur  ble  or   o  chil  hoo   c  n-
                   eosinophils. Fibrosis   n   necrosis   re  requent f n  ings.                                               cer, with 5-ye  r surviv  l r  tes excee  ing 98%.


                        Ho  gkin    ise  se  (Figs.  22.15    n    22.16)  is  consi  ere                                           In the e  rly st  ges o  Ho  gkin   ise  se, exten  e   f el   irr  -
                       istinct clinic  l entity. A   i  gnosis is       e pri    rily by                                         i  tion only w  s previously  the st  n    r   ther  py.  Rel  pse


                   ex    in  tion o  histology sections o  ly  ph no  es.                                                      occurre   in   pproxi    tely one thir   o  p  tients.   o    y, co  -
                        Ho  gkin   ise  se is ch  r  cterize   by    persistent   e ect in                                     bine      o    lity  tre  t  ent  with    bbrevi  te    che  other  py


                   the cellul  r i    unity with   bnor    lities in    ly  phocytes,                                            n    li  ite    irr    i  tion  h  s  re  uce    the  rel  pse  r  te,  but
                                                                                                                               r    i  tion ther  py incre  ses the risks o     secon   c  ncer.

                                                                                                                                    Zev  lin is the f rst   n   only r    ioi    unoconjug  te (RIC)

                                                                                                                               to be   pprove   by the U.S. FDA  or the tre  t  ent o  CD20-

                                                                                                                               positive rel  pse   or re r  ctory, low-gr    e,  ollicul  r, or tr  ns-

                                                                                                                                or  e   B-cell NHL, inclu  ing rituxi    b-re r  ctory  ollicul  r

                                                                                                                               NHL. T e toxicity o  this tre  t  ent is pri    rily he    tologic  l.

                                                                                                                               Approxi    tely 20% o  p  tients require tr  ns usions o  pl  te-

                                                                                                                               lets   n   RBCs. Severe neutropeni  , thro  bocytopeni  ,   n

                                                                                                                                 ne  i   occur in up to 30% o  tre  te   p  tients. Docu  ente

                                                                                                                               re  issions r  nge  ro   3 to 5+ ye  rs  ollowing tre  t  ent.

                                                                                                                                    T e tre  t  ent o      v  nce   Ho  gkin   ise  se w  s i  prove

                                                                                                                               consi  er  bly with the intro  uction o  co  bin  tion che  o-

                                                                                                                               ther  py—initi  lly    echloreth    ine,  vincristine,  proc  r-

                                                                                                                               b  zine,    n    pre  nisone  (MOPP)    n    l  ter  the  ABV   or

                                                                                                                               o     oxorubicin,  bleo  ycin,  vinbl  stine,    n        c  rb  zine
                   FIGURE  22.15  Ho  gkin  ly  phocyte–rich  “cl  ssic”  Ho  gkin
                     ise  se.  T e  b  ckgroun    is  pri    rily  ly  phocytes  with  Ree  -                                  (ABVD). P  tients receiving these ther  pies h  ve    20% to

                   Sternberg cells. (Reprinte    ro   Greer JP, et   l. Wintrobe’s Clinical                                    25% rel  pse r  te. Esc  l  te     oses o  f rst-line che  other  py

                   Hematology,  11th  e  , Phil    elphi  , PA:  Lippincott Willi    s  &                                        n   consoli    tion r    i  tion ther  py h  ve lowere   the rel  pse

                   Wilkins, 2004, with per  ission.)                                                                           r  te but without i  prove  ent in surviv  l.
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