1. CHRONIC MYELOPROLIFERATIVE DISORDER

2. TYPES 1. Chronic Myelogenous Leukemia 2. Polycythemiavera 3. Essential Thrombocytosis 4. Primary Myelofibrosis

3. CHRONIC MYELOGENOUS LEUKEMIA

5. Philadelphia Chromosome

6. Clinical Course Insidious Mild-Moderate Anemia Microcytic to MacrocyticanemiaHypermetabolicstate Organomegaly ExtramedullaryHematopoiesis Hepatosplenomegaly Mild Lymphadenopathy

7. Clinical Course Median survival is 3 years 50% enter an Accelerated Phase Increasing anemia, thrombocytopenia, striking basophilia 6-12 months later AML - Blastic crisis 50% Blast crisis

8. PBS Marked Leukocytosis Predominantly pmns, metamyelocytes, myelocytes Less 10% are Myeloblast Eosinophilia & Basophilia Thrombocytosis DESCRIPTION

9. DESCRIPTION BM Hypercellular 100% Composed mostly Maturing Granulocytic precursors

10. PBS- CML

11. ExtramedullaryHematopoiesis Hepatosplenomegaly Mild Lymphadenopathy DESCRIPTION

13. Treatment BCR-ABL kinase inhibitors May not prevent progression to crisis Allogenic bone marrow transplant Most effective Tx

14. Polycythemiavera Clonal D/O of pluripotent stem cell Undetectable levels of eryhtropoietin Increase in Myeloid Stem cells With progression  Lead to 1. myelofibrosis 2. Leukemic transformation

15. Polycythemiavera Late Middle age group blood Viscosity  Vascular stasis  Thrombotic tendency & Hemorrhagic diasthesis Gout , Pain on affected organ Death : 30% thrombotic complications – brain, heart 5-10% Bleeding

16. DIAGNOSIS : All 3 or 2 of Major + 2 Minor MAJOR Increased Total Erythrocyte Volume - Males > 36ml/Kg - Females > 32ml/Kg Normal Arterial O2 saturation > 92% Splenomegaly MINOR Thrombocytosis > 400 x 10 9/L Leukocytosis > 12 x 10 9 /L Increased NAP Increased Serum Vit B12 > 900 ug/L