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Myelodysplastic Syndromes and



                                                 24                          Myelodysplastic/Myeloproliferative


                     CHAPTER


                                                                             Neoplasms


















                                KEY TERMS






                   chronic m yelom onocytic leukem ia                                      myelodysplastic syndromes (MDSs)                                        refractory anem ia with excess blasts
                   idiopathic cytopenia of undeterm ined                                   myeloproliferative neoplasm s                                               (RAEB-1 and RAEB-2)

                       signi  cance (ICUS)                                                     (MPNs)                                                              refractory anem ia with ring sidero-
                   internuclear bridging (INB)                                             refractory anem ia (RA)                                                     blasts (RARSs)








                                LEARNING OUTCOMES






                   Classi  cation                                                                                              ■   Compare the laboratory features of speci  c types of MDSs.


                   ■   Describe the comparative characteristics of the French-American-                                        ■   Distinguish  between  agranular  blasts,  granular  blasts,  and

                       British (FAB) and World Health Organization (WHO) classi  cation of                                         promyelocytes.

                       myelodysplastic syndromes (MDSs) and myelodysplastic/myelopro-                                          ■   Calculate the percentage of myeloblasts in the bone marrow.

                       liferative neoplasms (MDS/MPN).                                                                         Myelodysplastic syndromes/ myeloproliferative


                   Pathophysiology                                                                                             neoplasms


                     Explain the pathophysiology of MDSs.                                                                      ■   Describe the unique features and laboratory characteristics of

                                                                                                                                   chronic myelomonocytic leukemia (CMML).
                   Etiology                                                                                                      Distinguish between MDS and acute leukemia



                   ■   Explain the causes or predisposing factors of primary and secondary
                       MDSs.                                                                                                   Treatment

                                                                                                                                 Explain the forms of treatment and supportive care for the MDSs.
                   Epidemiology


                   ■   Describe the age and gender distribution of MDSs.                                                       Relationship of cytogenetics to prognosis

                                                                                                                               ■   Discuss factors that can affect prognosis in the MDSs, including FAB
                   Chromosomal abnormalities                                                                                       classi  cation and karyotype.


                   ■   Brie  y describe the causes, types, and consequences of chromo-                                           Compare  the  parameters  used  in  the  International  Scale  for

                       somal abnormalities in MDSs.                                                                                Prognosis.

                   ■   List the incidence of chromosomal abnormalities.

                   ■   Describe the relationship of cytogenetic   ndings to prognosis in                                       Case studies

                       MDSs.                                                                                                     Analyze the patient history, clinical signs and symptoms, and labo-
                                                                                                                                   ratory data for the stated case studies, answer the related critical
                   Clinical signs and symptoms                                                                                     thinking questions, and conclude the most likely diagnosis.



                   ■   Explain the clinical signs and symptoms of MDSs.

                   Laboratory manifestations



                   ■   Itemize the cellular alterations, with an emphasis on the prominent                                        NOTE:
                                                                                                                                      Indicates MLT and MLS core content
                                                                                                                                  ■
                       features and additional hematological features in the four sub-                                              Indicates MLT (optional) and MLS advanced content
                       groups of MDS/MPN.
























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