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Myelodysplastic Syndromes

Myelodysplastic Syndromes. Austin Kulasekararaj. Background and Why?. Described in 1900 Defined as MDS only in 1982-abnormal clone (don’t think it spreads like breast cancer) No interest in studying MDS New research, novel treatments and more emphasis on disease biology.

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Myelodysplastic Syndromes

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  1. Myelodysplastic Syndromes Austin Kulasekararaj

  2. Background and Why? • Described in 1900 • Defined as MDS only in 1982-abnormal clone (don’t think it spreads like breast cancer) • No interest in studying MDS • New research, novel treatments and more emphasis on disease biology

  3. What does the term "myelodysplastic" actually mean? • Myelo = marrow • Dysplastic = abnormal or just gone bad or funny looking Bone marrow cells fail to make enough healthy blood cells- quantity and quality is affected. • Crowding out remaining normal cells (Cancer)

  4. Red Blood Cells 20 trillion

  5. MDS features All people with MDS have two things in common • Low count for at least 1 blood cell type (cytopenia) • Bone marrow and blood contain blood cells with an abnormal shape, size, or look.

  6. Symptoms of MDS Asymptomatic – abnormal blood count Fatigue, lack of energy and shortness of breath - caused by anaemia (low red cells) Bruising and bleeding - caused by low platelet cell count Infection - due to low numbers and/or poorly functioning white cells

  7. Patients Prior exposure to chemo and or radiotherapy, but 90% do not have any known exposures Median age is @ 72 years Clinical Course AML 25% MDS 50% Infection, Bleeding and treatment complications Unrelated causes 25%

  8. Ouch Bone marrow aspirate and trephine Diagnostic Tests Blood film/Aspirate Cytogenetics Flow Cytometry Trephine

  9. Blasts (Type 1 and 2) Morphology of MDS 5q- RARS

  10. Beware of mimics, imposters, copycats and imposters • Vitamin deficiency • Other related disorders (aplastic anaemia) • ICUS (Idiopathic Cytopenia of Unknown Significance) • Nutritional and pharmacological toxicities • Immune related conditions (lupus, rheumatoid and crohns/ulcerative colitis)

  11. RAEB-t ? MDS-not a single entity? PNH AML AA MDS MDS AA RAEB 1/2 RA/RARS CMML MPD/MDS MPN (BCR-ABL neg) AML Low Risk High Risk Clone size RCMD Hypo MDS 5q- CMML aCML MDS/MPN-U JMML AML RA /RARS 5q- JAK2+ RARS-T Pre-MDS/NY/NQ MDS/ ICUS/ IDUS Normal Time (years)

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