Chronic Myeloproliferative Disorder

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Chronic Myeloproliferative Disorder

  1. 1. CHRONIC MYELOPROLIFERATIVE DISORDER<br />
  2. 2. TYPES<br />1. Chronic Myelogenous Leukemia<br />2. Polycythemiavera<br />3. Essential Thrombocytosis<br />4. Primary Myelofibrosis<br />
  3. 3. CHRONIC MYELOGENOUS LEUKEMIA<br />
  4. 4. DESCRIPTION<br /><ul><li>Primarily Adults ( 25-60 y/o )</li></ul>Peak age 4th – 5th <br />> 90% Philadelphia Chromosome<br />t(9,22)(q34;q11)<br />ABL-BCR gene translocation<br />Affect Pluripotent stem cell<br />Net effect is Unregulated cell division & inhibition of apoptosis<br />
  5. 5. Philadelphia Chromosome<br />
  6. 6. Clinical Course<br />Insidious <br />Mild-Moderate Anemia<br />Microcytic to MacrocyticanemiaHypermetabolicstate<br />Organomegaly<br />ExtramedullaryHematopoiesis<br />Hepatosplenomegaly<br />Mild Lymphadenopathy<br />
  7. 7. Clinical Course<br />Median survival is 3 years<br />50% enter an Accelerated Phase<br />Increasing anemia, thrombocytopenia, striking basophilia<br />6-12 months later AML - Blastic crisis<br />50% Blast crisis<br />
  8. 8. PBS<br />Marked Leukocytosis<br />Predominantly pmns, metamyelocytes, myelocytes<br />Less 10% are Myeloblast<br />Eosinophilia & Basophilia<br />Thrombocytosis<br />DESCRIPTION<br />
  9. 9. DESCRIPTION<br />BM Hypercellular 100%<br /> Composed mostly Maturing Granulocytic precursors<br />
  10. 10. PBS- CML<br />
  11. 11. ExtramedullaryHematopoiesis<br />Hepatosplenomegaly<br />Mild Lymphadenopathy<br />DESCRIPTION<br />
  12. 12.
  13. 13. Treatment<br />BCR-ABL kinase inhibitors<br />May not prevent progression to crisis<br />Allogenic bone marrow transplant<br />Most effective Tx<br />
  14. 14. Polycythemiavera<br />Clonal D/O of pluripotent stem cell<br />Undetectable levels of eryhtropoietin<br />Increase in Myeloid Stem cells<br />With progression  Lead to<br />1. myelofibrosis<br />2. Leukemic transformation<br />
  15. 15. Polycythemiavera<br /> Late Middle age group<br /> blood Viscosity  Vascular stasis  Thrombotic tendency & Hemorrhagic diasthesis<br />Gout , Pain on affected organ<br />Death :<br />30% thrombotic complications – brain, heart<br /> 5-10% Bleeding<br />
  16. 16. DIAGNOSIS : <br />All 3 or 2 of Major + 2 Minor<br />MAJOR<br />Increased Total Erythrocyte Volume<br /> - Males > 36ml/Kg<br /> - Females > 32ml/Kg<br />Normal Arterial O2 saturation > 92%<br />Splenomegaly<br />MINOR<br />Thrombocytosis > 400 x 10 9/L<br />Leukocytosis > 12 x 10 9 /L<br />Increased NAP<br />Increased Serum Vit B12 > 900 ug/L<br />

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