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  • image Myelodysplastic syndromes (MDS) primarily affect elderly adults, with median age at diagnosis of 76 years.
  • image MDS are associated with environmental, occupational, and therapeutic exposures to chemicals or radiation.
  • image The clonal population of cells manifested as MDS results from enhanced self-renewal of a hematopoietic stem cell or acquisition of self-renewal in a progenitor cell, increased proliferative capacity in the abnormal clone, impaired cell differentiation, evasion of immune regulation, and antiapoptotic mechanisms in the disease-sustaining cell.
  • image Most patients with MDS present with fatigue and lethargy or symptoms related to anemia-induced tissue hypoxia.
  • image The prognosis of patients with MDS is variable. Overall survival time ranges from a few months to several years and can be estimated with the International Prognostic Scoring System (IPSS) or International Prognostic Scoring System—Revised (IPSS-R).
  • image The primary goal of therapy is hematologic improvement for low-risk patients and alteration in the natural course of the disease for high-risk patients. Palliation of symptoms and improvement in quality of life are goals of therapy for all patients.
  • image Current guidelines recommend erythropoietin (EPO) or darbepoetin for management of anemia in patients with MDS.
  • image Hypomethylating agents are appropriate for patients with low-risk and intermediate-1-risk MDS with clinically significant neutropenia or thrombocytopenia, patients with anemia who are unlikely to respond to or have not responded to a trial of EPO, and patients who qualified for and failed immunosuppressive therapy.
  • image Antithymocyte globulin is appropriate treatment for low or intermediate-1 IPSS risk, human leukocyte antigen DR15 positive expressing MDS in patients with symptomatic anemia that is unlikely to respond to erythropoietic agents.
  • imageLenalidomide is the recommended initial treatment for low-risk 5q- syndrome accompanied by symptomatic anemia.
  • image Allogeneic hematopoietic stem cell transplantation offers potentially curative therapy to patients with MDS who have a donor and are healthy enough for the procedure.

On completion of the chapter, the reader will be able to:

  1. Explain the pathophysiology of myelodysplastic syndromes.

  2. Discern the class of chemotherapeutic agents that contributed to the development of myelodysplastic syndromes given cytogenetic analysis of the bone marrow.

  3. Identify symptoms a patient may present with that are consistent with a diagnosis of myelodysplastic syndrome.

  4. Describe testing that should be done to diagnose a patient with myelodysplastic syndrome.

  5. Calculate the International Prognostic Scoring System risk score for a patient with myelodysplastic syndrome when given percentage of bone marrow blasts, cytogenetics, and number of cytopenias.

  6. Discern the goals of therapy for a patient with myelodysplastic syndrome based on age, presence of symptoms, International Prognostic Scoring System risk score, and eligibility for allogeneic hematopoietic stem cell transplantation.

  7. Recommend appropriate supportive care for a patient with symptomatic myelodysplastic syndrome.

  8. Compare the rates of complete response, hematologic improvement, and overall survival of therapeutic options used to treat myelodysplastic syndromes.

  9. Recommend an appropriate therapeutic regimen for a patient given his or her International Prognostic Scoring System risk category, age, cytogenetic information, and eligibility for allogeneic hematopoietic stem cell transplantation.

  10. Determine the likelihood of response to erythropoiesis-stimulating agents based on erythropoietin level and ...

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