Page 495 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )
P. 495

CHAPTER 24  ■  Myelodysplastic Syndromes and Myelodysplastic/Myeloproliferative Neoplasms                                                    479







                       TABLE         24.3       Fourth Edition (2003) WHO Criteria MDSs





                       MDS Subtype                                                Peripheral Blood                                            Bone Marrow



                       Refractory anemia (RA)*                                    Anemia                                                      Unilineage dysplasia ≥10% in one myeloid line


                                                                                  <1% blasts                                                  <5% blasts

                                                                                                                                              <15% ring sideroblasts


                       Refractory anemia with ring                                Anemia                                                      Erythroid dysplasia only ≥15% ring sideroblasts

                       sideroblasts (RARS)


                                                                                  No blasts                                                   <5% blasts

                       Refractory cytopenias with                                 Cytopenias                                                  Dysplasia in ≥10% of cells in two myeloid cell lines

                       multilineage dysplasia (RCMD)


                                                                                  No or rare blasts                                           No Auer rods


                                                                                  <1 × 10 /L monocytes                                        <5% blasts in marrow
                                                                                             9

                                                                                  No Auer rods                                                No Auer rods

                                                                                                                                              ±15% ring sideroblasts


                       Refractory anemia with excess                              Cytopenias                                                  Unilineage or multilineage dysplasia

                       blasts type 1 (RAEB-1)


                                                                                  <5% blasts                                                  5%–9% blasts


                                                                                  No Auer rods                                                No Auer rods

                                                                                  <1 × 10 /L monocytes
                                                                                             9

                       Refractory anemia with excess                              Cytopenias                                                  Unilineage or multilineage dysplasia

                       blasts type 2 (RAEB-2)


                                                                                  <5%–19% blasts                                              10%–19% blasts


                                                                                  Auer rods ± <1 × 10 /L monocytes                            Auer rods ±
                                                                                                                9
                       MDS associated with isolated                               Anemia                                                      <5% blasts

                       del (5q)


                                                                                  Normal or elevated platelet count                           Anemia, hypolobulated megakaryocytic anemia

                                                                                                                                              isolated 5q31 chromosome deletion


                                                                                  <1% blasts

                       Childhood MDS, including                                   Pancytopenia                                                <5% marrow red blood cell blasts

                       refractory cytopenia of childhood

                       (provisional)


                                                                                                                                              Usually hypocellular marrow


                       MDS, unclassi  able (MDS-U)                                Cytopenias                                                  Does not   t other categories


                                                                                  ≤1% blasts                                                  Dysplasia and <5% blasts

                                                                                                                                              If no dysplasia, MDS-associated karyotype



                       *This category is refractory cytopenias with unilineage dysplasia (RCUD) refractory anemia. The category includes refractory anemia, refractory neutropenia,
                       and refractory thrombocytopenia.


                       MDS, myelodysplastic syndrome.






                   Platelet Abnorm alities                                                                                     bulge o  the cell   e  br  ne, h  s been observe  . Incre  se

                   T e   eg  k  ryocyte popul  tion     y be   ecre  se  , nor    l, or                                        nu  bers o  these   typic  l pl  telets c  n be observe   in the

                   incre  se  . Micro  eg  k  ryocytes,   ononucle  r   eg  k  ryo-                                                jority o  p  tients with MDS. Nor    l pl  telet   orphol-

                   cytes,   ultiple s    ll sep  r  te   nuclei,   n   gi  nt gr  nules c  n                                   ogy     y be observe   in p  tients with RARS. T e nu  ber

                   be seen in the peripher  l bloo  . L  rge biz  rre pl  telets   re                                          o    typic  l pl  telets is neg  tively correl  te   with the periph-

                       requent f n  ing in the peripher  l bloo  . A   istinct sub-                                            er  l pl  telet counts in MDS. T e   typic  l pl  telets   ost likely

                   popul  tion  o   pl  telets  in  MDS,  which  by  ph  se-contr  st                                          re  ect      tur  tion    isturb  nces  o     eg  k  ryocytopoiesis.

                     icroscopic  ex    in  tion  see    to  h  ve      b  lloon-sh  pe                                         Unless   ssoci  te   with recent cytotoxic ther  py,   n incre  se
   490   491   492   493   494   495   496   497   498   499   500