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CHAPTER 22  ■  Lymphoid and Plasma Cell Neoplasms                                  427




                                                                                                                               types. R  re  or  s o  ly  pho     inclu  e Burkitt’s ly  pho

                        BOX  22.3                                                                                                n     ycosis  ungoi  es,    v  ri  nt o  Séz  ry syn  ro  e.

                                                                                                                                    T e  two  conte  por  ry  ly  pho      cl  ssif c  tion  sys-

                                                                                                                               te  s    re  the  Revise    Europe  n-A  eric  n  Ly  pho
                     Examples of Lymphoid Neoplasms                                                                            (REAL) Cl  ssif c  tion   n   the Worl   He  lth Org  niz  tion


                                                                                                                               (WHO)  Cl  ssif c  tion  o     u  ours  o   the  H  e    topoietic
                     B-CELL NEOPLASMS

                     Precursor B-cell neopl  s                                                                                   n   Ly  phoi     issues,  ourth e  ition. T ere   re   pproxi-
                                                                                                                                   tely  30    i  erent  ly  pho        bnor    lities,  inclu  ing

                     ■    Precursor B-ly  phobl  stic leuke  i  /ly  pho     (pre-                                             entities   ef ne   by    co  bin  tion o    orphologic  l, i    un-

                          cursor B-cell   cute ly  phobl  stic leuke  i  )                                                     ophenotypic  l, genetic,   n   clinic  l  e  tures (see     bles 22.6


                          M  ture (peripher  l) B-cell neopl  s  s with leuke  ic                                                n    22.7).  T e  i  port  nce  o   the  v  rious   e  tures    i  ers

                     present  tion                                                                                               epen  ing on the   i  erent types o  ly  pho    s.
                                                                                                                                    Te REAL   n   WHO cl  ssif c  tions inclu  e Ho  gkin   is-

                     ■    Chronic ly  phocytic leuke  i  /B-cell SLL                                                           e  se   n   NHL. T e ter   Ho  gkin ly  pho     is pre erre

                     ■    B-cell proly  phocytic leuke  i                                                                      bec  use o  the origin o  the Reed-Sternberg cell in   l  ost   ll

                     ■    H  iry cell leuke  i                                                                                 c  ses o  Ho  gkin   ise  se   s the B ly  phocytes. T e WHO

                     ■    Pl  s     cell neopl  s  s                                                                           cl  ssif c  tion   lso inclu  es pl  s     cell neopl  s  s within the


                          M  ture (peripher  l) B-cell neopl  s  s no  e b  se                                                 c  tegory o  B-cell neopl  s  s. Sep  r  te sections o  the WHO
                                                                                                                               cl  ssif c  tion inclu  e i    uno  ef ciency-  ssoci  te   ly  pho-

                     ■    Di  use l  rge B-cell ly  pho                                                                        proli er  tive   isor  ers,   cute   yeloi   leuke  i  s,   yelo  ys-

                     ■    Burkitt ly  pho    /leuke  i                                                                         pl  stic syn  ro  es,   n   chronic   yeloproli er  tive   isor  ers.

                                                                                                                                    Within the group o  NHLs, ly  phobl  stic neopl  s  s   re
                     T- AND NK-CELL NEOPLASMS

                     Precursor   -cell neopl  s                                                                                sep  r  te    ro       ture B-   n     -cell neopl  s  s. Both the
                                                                                                                                   ture B-   n     -cell neopl  s  s   re subcl  ssif e   into those

                     ■    Precursor   -ly  phobl  stic leuke  i  /ly  pho     (pre-                                            th  t   re   s  ollows:

                          cursor   -cell   cute ly  phobl  stic leuke  i  )
                                                                                                                               ■   Pre  o  in  ntly   isse  in  te   or leuke  ic   t present  tion
                          M  ture (peripher  l)   -cell neopl  s  s with leuke  ic                                             ■   Pri    rily extr  no    l

                     present  tion                                                                                             ■   Pre  o  in  ntly ly  ph no  e–b  se   entities



                     ■      -cell proly  phocytic leuke  i                                                                          As     v  nces   re       e in he    top  thology, ch  nges will

                     ■    A  ult   -cell ly  pho    /leuke  i   (H  LV 1+)                                                     be incorpor  te   into the WHO cl  ssif c  tion syste  . Precise

                     ■    Mycosis  ungoi  es/Séz  ry syn  ro  e                                                                  i  gnostic subcl  ssif c  tion is i  port  nt to       inister e  ec-

                                                                                                                               tive ther  py.
                     HODGKIN LYMPHOMA (HODGKIN DISEASE)



                     ■    No  ul  r ly  phocyte pre  o  in  nt Ho  gkin ly  pho                                                Epidemiology

                     ■    Cl  ssic  l Ho  gkin ly  pho
                                                                                                                               Ly  pho    s  h  ve  been    escribe    in    ll  r  ces    n    ethnic

                                                                                                                               groups. Ly  pho     is the thir     ost co    on  or   o  c  n-

                                                                                                                               cer     ong chil  ren  ollowing leuke  i  s   n   br  in tu  ors.
                   Classi  cations
                                                                                                                               Ho  gkin ly  pho     or Ho  gkin’s   ise  se,   ost co    only

                   Di  gnosis   n   subcl  ssif c  tion o  ly  pho     h  ve ch  nge                                               ects chil  ren   ge   15   n   ol  er. T ree subtypes o  Ho  gkin

                     r      tic  lly over ti  e. Cl  ssic  lly, the     jor  or  s o      lig-                                 ly  pho     exist in chil  ren. T e subtype, No  ul  r sclerosis

                   n  nt ly  pho    s   re   ivi  e   into Ho  gkin   n   non-Ho  gkin                                         (NS     ects 70% o  chil  ren who   re   i  gnose   with Ho  gkin










                       TABLE         22.6       Immunohistological Features of Selected B-Cell Neoplasms




                                                                                       Surface Membrane                                    Marker



                       Type of Neoplasm                                                SIg                                                 CD5                    CD10                      CD23                      CD43



                       Chronic lymphocytic leukemia                                      +                                                    +                       −                         +                         +


                       Hairy cell leukemia                                               +                                                    −                       −                         −                         +


                       Burkitt lymphoma                                                  +                                                    −                       +                         −                         −


                       SIg, surface immunoglobulin.


                       Source: Handin RI, et al. (eds.), Blood: Principles and Practice of Hematology, 2nd ed, Philadelphia, PA: Lippincott Williams & Wilkins, 2003:75.
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