C H R O N I C M Y E L O P R O L I F E R A T I V E D I S O R D E R
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Transcript of C H R O N I C M Y E L O P R O L I F E R A T I V E D I S O R D E R
CHRONIC MYELOPROLIFERATIVE
DISORDER
TYPES1. Chronic Myelogenous Leukemia
2. Polycythemia vera3. Essential Thrombocytosis
4. Primary Myelofibrosis
CHRONIC MYELOGENOUS LEUKEMIA
DESCRIPTIONPrimarily Adults ( 25-60 y/o )
Peak age 4th – 5th
> 90% Philadelphia Chromosome
t(9,22)(q34;q11)ABL-BCR gene translocationAffect Pluripotent stem cell
Net effect is Unregulated cell division & inhibition of apoptosis
PHILADELPHIA CHROMOSOME
Clinical Course Insidious Mild-Moderate Anemia
Microcytic to Macrocytic anemiaHypermetabolic state
Organomegaly Extramedullary Hematopoiesis Hepatosplenomegaly Mild Lymphadenopathy
CLINICAL COURSE Median survival is 3 years 50% enter an Accelerated PhaseIncreasing anemia, thrombocytopenia, striking basophilia
6-12 months later AML - Blastic crisis
50% Blast crisis
PBSMarked LeukocytosisPredominantly pmns, metamyelocytes, myelocytes
Less 10% are MyeloblastEosinophilia & BasophiliaThrombocytosis
DESCRIPTION
DESCRIPTION BM Hypercellular 100%
Composed mostly Maturing Granulocytic precursors
PBS- CML
Extramedullary Hematopoiesis Hepatosplenomegaly Mild Lymphadenopathy
DESCRIPTION
FEATURE LEUKEMOIDREACTION
CHRONIC MYELOGENOUSLEUKEMIA
1. WBC < 50 x 109/L > 50 x 109/L
2. Toxic Granulation
Positive Negative
3. Basophilia Absent Greater basophia
4. Cell Bands-prominent
Immature all stages, particularly myelocyte
5. LAP High > 100 Low < 10
6. Splenomegaly Absent Present
Philadelphia Chromosome
Absent Present
TREATMENT BCR-ABL kinase inhibitors
May not prevent progression to crisis
Allogenic bone marrow transplantMost effective Tx
POLYCYTHEMIA VERA Clonal D/O of pluripotent stem cell
Undetectable levels of eryhtropoietin
Increase in Myeloid Stem cells With progression Lead to
1. myelofibrosis2. Leukemic transformation
POLYCYTHEMIA VERA 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
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
MINORThrombocytosis > 400 x 10 9/LLeukocytosis > 12 x 10 9 /L Increased NAP Increased Serum Vit B12 > 900 ug/L