TY  - CHAP
M1  - Book, Section
TI  - Myelodysplastic Syndromes
A1  - Rowe, Julie H.
A1  - Gonzalez, Anneliese O.
A1  - Jafri, Syed H.
A1  - Cen, Putao
A1  - Kanaan, Zeyad
A1  - Amato, Robert J.
A1  - Rios, Adan
A1  - El-Osta, Hazem
A1  - Mohlere, Virginia
Y1  - 2019
N1  - 
T2  - Hematology-Oncology Clinical Questions
AB  - Table  Graphic  Jump  Location|Download  (.pdf)|PrintKey  conceptMDSs  are  a  group  of  heterogeneous  myeloid  hematopoietic  disorders  characterized  by  ineffective  hematopoiesis  and  increased  risk  of  transformation  to  acute  myelogenous  leukemia  (AML).  Most  patients  with  MDS  die  of  causes  related  to  the  disease.  The  median  age  of  patients  with  MDS  is  70–75  years.1Clinical  scenarioA  71-year-old  man  is  admitted  to  a  hospital  with  generalized  weakness  and  dyspnea  on  exertion.  On  physical  examination,  the  abdominal  reveals  no  organomegaly.  His  hemoglobin  level  is  6  g/dL,  and  the  rest  of  his  blood  work  is  normal.  There  is  no  obvious  evidence  of  blood  loss.  A  bone  marrow  examination  shows  normocellular  marrow  with  dysplastic  changes  in  the  megakaryocytes  and  erythroid  series.  Increased  promyelocytes  with  granules  (14.6%),  eosinophils  (5.6%),  and  basophils  (7%)  are  observed.  Karyotype  is  normal  (46,XX).  Diagnosis  is  MDS  with  eosinophilia  and  basophilia  (refractory  cytopenia  with  multilineage  dysplasia).Action  itemsReview  whether  a  patient  diagnosed  with  MDS  meets  minimal  diagnostic  criteria2:Stable  cytopenia  (6  months,  or  2  months  if  there  is  a  specific  MDS  karyotype  or  bilineage  dysplasia)Exclusion  of  other  disorders  as  cause  of  cytopenia  and/or  dysplasia  or  bothAt  least  1  of  the  3  MDS-related  (decisive)  criteria:  Dysplasia  (>10%  in  ≥1  of  3  major  bone  marrow  lineages)A  blast  count  of  5%–19%Specific  karyotype,  such  as  del(5q),  del(20)q,  +8,  or  –7del  (7q)Co-criteria  help  to  confirm  diagnosis:Abnormal  bone  marrow  immune  histology,  immunohistochemistryAbnormal  flow  cytometryAbnormal  CD34  expression  FibrosisMyeloid  clonalityDysplastic  megakaryocytesDiscussionMDS  is  usually  suspected  when  there  is  cytopenia  in  a  routine  peripheral  blood  count.  A  bone  marrow  aspiration  and  biopsy  follows,  with  a  manual  count  of  blasts,  which  is  essential  for  risk  assessment.  Cytogenetic  analysis  assists  in  predicting  risk  and  selecting  therapy.  Risk  is  calculated  using  the  International  Prognostic  Scoring  System  (IPSS).  Patients  with  low  and  intermediate-1  (INT-1)  scores  are  treated  to  improve  transfusion  needs.  Patients  with  higher  scores,  intermediate-2  (INT-2)  or  higher,  are  treated  with  interventions  patterned  after  AML  therapy.  The  revised  IPSS  score  (IPSS-R)  includes  a  new  cytogenetic  risk  classification  that  divides  patients  into  5  categories.  It  is  used  more  for  prognosis  than  for  deciding  therapy,  since  all  the  approved  treatments  for  MDS  have  been  approved  in  clinical  trials  using  the  IPSS.PearlIPSS  criteria  are  used  to  decide  therapy  and  IPSS-R  criteria  to  evaluate  prognosisReferencesMontalban-Bravo  G,  Garcia-Manero  G.  Myelodysplastic  syndromes:  2018  update  on  diagnosis,  risk-stratification  and  management.  Am  J  Hematol  2018;93:129-47.Valent  P,  Horny  HP,  Bennett  JM,  et  al.  Definitions  and  standards  in  the  diagnosis  and  treatment  of  the  myelodysplastic  syndromes:  consensus  statements  and  report  from  a  working  conference.  Leuk  Res  2007;31(6):727-36.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - hemonc.mhmedical.com/content.aspx?aid=1162981522
ER  -