This document summarizes four types of chronic myeloproliferative disorders: chronic myelogenous leukemia, polycythemia vera, essential thrombocytosis, and primary myelofibrosis. It provides details on chronic myelogenous leukemia such as its description, clinical course, Philadelphia chromosome translocation, and treatment options including BCR-ABL kinase inhibitors and allogenic bone marrow transplant. For polycythemia vera, it notes the clonal disorder of pluripotent stem cells, symptoms, complications including thrombosis and bleeding, and diagnostic criteria.

1. CHRONIC MYELOPROLIFERATIVE DISORDER

2. TYPES1. Chronic Myelogenous Leukemia2. Polycythemiavera3. Essential Thrombocytosis4. Primary Myelofibrosis

3. CHRONIC MYELOGENOUS LEUKEMIA

4. DESCRIPTIONPrimarily Adults ( 25-60 y/o )Peak age 4th – 5th > 90% Philadelphia Chromosomet(9,22)(q34;q11)ABL-BCR gene translocationAffect Pluripotent stem cellNet effect is Unregulated cell division & inhibition of apoptosis

5. Philadelphia Chromosome

6. Clinical CourseInsidious Mild-Moderate AnemiaMicrocytic to MacrocyticanemiaHypermetabolicstateOrganomegalyExtramedullaryHematopoiesisHepatosplenomegalyMild Lymphadenopathy

7. Clinical CourseMedian survival is 3 years50% enter an Accelerated PhaseIncreasing anemia, thrombocytopenia, striking basophilia6-12 months later AML - Blastic crisis50% Blast crisis

8. PBSMarked LeukocytosisPredominantly pmns, metamyelocytes, myelocytesLess 10% are MyeloblastEosinophilia & BasophiliaThrombocytosisDESCRIPTION

9. DESCRIPTIONBM Hypercellular 100% Composed mostly Maturing Granulocytic precursors

10. PBS- CML

11. ExtramedullaryHematopoiesisHepatosplenomegalyMild LymphadenopathyDESCRIPTION

13. TreatmentBCR-ABL kinase inhibitorsMay not prevent progression to crisisAllogenic bone marrow transplantMost effective Tx

14. PolycythemiaveraClonal D/O of pluripotent stem cellUndetectable levels of eryhtropoietinIncrease in Myeloid Stem cellsWith progression  Lead to1. myelofibrosis2. Leukemic transformation

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

16. DIAGNOSIS : All 3 or 2 of Major + 2 MinorMAJORIncreased Total Erythrocyte Volume - Males > 36ml/Kg - Females > 32ml/KgNormal Arterial O2 saturation > 92%SplenomegalyMINORThrombocytosis > 400 x 10 9/LLeukocytosis > 12 x 10 9 /LIncreased NAPIncreased Serum Vit B12 > 900 ug/L