Myelodysplastic Syndromes

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julieaburch
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87383
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Myelodysplastic Syndromes
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2011-05-24 13:54:14
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Myelodysplastic Syndromes
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Myelodysplastic Syndromes
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  1. This is a heterogenous group of disorders characterized by: cytopenia of one or more lineages; dysplastic cellular appearance; abnormal or impaired cell function; and a variable predisposition to transform to acute myeloid leukemia. It is an insufficient number of funny looking cells that don't work well! There is genetic damage to hematopoietic stem cells, resulting in ineffective hematopoiesis -- the marrow is usually cellular, but many cells die in the marrow. The proliferative rate is often increased, with increased apoptosis, and impaired maturation and differentiation.
    Myelodysplastic Syndromes
  2. In Myelodysplastic Syndromes, there is an early increase in both proliferation and apoptosis. Later, there may be an "apoptotic escape:" increased ____________ may result in leukemic transformation. Increased apoptosis is multifactorial: increased pro-apoptotic cytokines in the marrow are present; increased expression of __________ occurs on hematopoietic precursors, which is proapoptotic.
    • Proliferation
    • fas (CD95)
  3. What syndrome is approximately twice as common as AML? This predominantly afflicts the older population, and median age is greater than 65 years. Risk increases with age, and this is rare in children <50, except when its therapy-related. Men are usually affected at a higher incidence than women. In the majority of cases there are no known predisposing factors.
    Myelodysplastic Syndromes
  4. What is the following clinical presentation generally associated with?:

    Many patients are asymptomatic, with cytopenia detected on routine laboratory tests. Common symptoms include: fatigue, weakness, dizziness, exacerbation of angina. Less common presentations include infections (primarily cutaneous) and bleeding (petechiae and easy bruising)
    Myelodysplastic Syndromes
  5. What laboratory values are associated with myelodysplastic syndromes?
    • Quantitative deficiencies in one or more cell types
    • Anemia: most common
    • Leukopenia
    • Thrombocytopenia
    • Pancytopenia
  6. What are the erythroid abnormalities associated with Myelodysplastic Syndromes (MDS)?
    • Anemia
    • Retic count inappropriately low
    • Usually normocytic or mildly macrocytic
    • Oval macrocytes common (may have elliptocytes, teardrop cells, or acanthocytes; may have basophilic stippling, Howell-Jolly bodies, nucleated RBCs)
  7. Identify the syndrome based on the blood smear below:
    Myelodysplastic Syndromes: note the anisocytosis and the oval macrocyte to the upper left of the lymphocyte
  8. These leukocyte abnormalities are seen in what syndrome?: granulocytopenia; nuclear changes -- decreased segmentation ("pseudo-Pelger-Huet" anomaly), abnormal chromatin condensation, nuclear sticks or ring-shaped nuclei; decreased or absent cytoplasmic granules
    Myelodysplastic Syndromes
  9. Identify the phenomenon seen in the blood smear below:
    Pseudo-Pelger-Huet Phenomenon (seen in Myelodysplastic Syndromes)
  10. The following platelet abnormalities are seen in what syndromes?: Thrombocytopenia; giant platelets, absent or abnormal granules, megakaryocyte fragments may be present
    Myelodysplastic Syndromes
  11. Identify the syndrome from the blood smear below:
    Myelodysplastic Syndromes: erythroid hyperplasia; nuclear-cytoplasmic asynchrony ("megaloblastoid appearance"); nuclear budding, multilobation, fragmentation or multinucleation; cytoplasmic vacuolization; ringed sideroblasts may be seen
  12. Identify the syndrome from the blood smear below:
    Myelodysplastic Syndrome
  13. Identify the phenomenon seen below:
    Refractory Anemia with Ringed Sideroblasts (seen in MDS)
  14. Identify the syndrome seen below:
    Myelodysplastic Syndromes
  15. Identify the syndromes seen below:
    MDS
  16. In this phenomenon seen in Myelodysplastic Syndromes, dysplastic changes are seen, but in one lineage only. By far the most common is Refractory Anemia (RA); Refractory Neutropenia (RN) is rarely seen, as well as Refractory Thrombocytopenia (RT)
    Refractory Cytopenia with Unilineage Dysplasia
  17. In the WHO Classification of MDS, this is, by definition, dysplasia that is restricted to the erythroid lineage. Cases with multilineage dysplasia but low blast counts are classified as RCMD: worse survival than cases with equivalent blast counts but pure erythroid dysplasia.

    This, is like RA, but with greater than 15% ringed sideroblasts
    • Refractory Anemia
    • RARS
  18. Cases of MDS with greater than 20% blasts are classified as what?
    AML
  19. This is categorized as dysplastic changes in 2 or more cell lines: usually erythroid plus granulocytic and/or megakaryocytic lineages. This requires dysplastic changes in greater than 10% of cells in 2 or more cell lines. Blasts must be <1% in blood, <5% in marrow. This has a shorter survival and greater progression to AML than cases with dysplasia limited to erythroid series.
    Refractory Cytopenia with Multilineage Dysplasia (RCMD)
  20. This is categorized as multilineage cytopenias and dysplastic changes present: more severe quantitative and morphologic abnormalities than RA. This is subdivided into a 1 & 2 based on percent blasts in blood and marrow, plus or minus Auer rods. This has a shorter survival; higher transformation to AML
    Refractory Anemia with Excess Blasts (RAEB)
  21. This syndrome predominantly occurs in older women. It is characterized by marked macrocytic anemia, with a platelet count that is normal to elevated. There is a deletion in the long arm of chromosome 5 as sole cytogenetic abnormality (deletion between q31-33). There is a long survival; and a rare transformation to AML
    MDS with Isolatated del(5q) Chromosome Abnormality (5q-Syndrome)
  22. How is Myelodysplastic Syndrome diagnosed?
    • Diagnosis of Exclusion
    • Must exclude: nutritional deficiency, alcohol abuse, myelosuppressive drugs, occupational exposure to toxic chemicals, or HIV and other infections
  23. The frequency of cytogenetic abnormalities varies with MDS subtype. In which subtype is it lowest in, and in which is it highest?
    • Lowest in RA
    • Highest in RARS
  24. There is a wide variety of karyotypic abnormalities in MDS. Balanced translocations, as seen in AML, are rare. In MDS, they are usually __________ abnormalities and what?
    • Numerical
    • Partial Chromosomal Deletions
  25. What are the three types of common cytogenetic abnormalities seen in MDS?
    • Interstitial Deletions
    • Chromosome Deletions
    • Chromosome Duplications
  26. What are the favorable cytogenetics in MDS?
    • Y
    • 5q
    • 20q
  27. What are the unfavorable cytogenetics in MDS?
    Chromosome 7 Abnormalities
  28. What are the three most common causes of death in MDS?
    • Infection -- most common
    • Bleeding
    • Leukemic Transformation
  29. What is the treatment for MDS?
    • Supportive: transfusions, antibiotics for infections, growth factors helpful in some patients
    • Cytogenetic Chemotherapy: young patients with aggressive variants
    • Allogenic Stem Cell Transplant: considered for younger patients

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