Chronic Myelogenous Leukemia

( Chronic Granulocytic Leukemia )

 I. Introduction :
Chronic Myelogenous Leukemia ( CML )
- Belongs to Myeloproliferative Disorders
- Adult & elderly
- Relatively slow clinical progression
- Relatively better live expectancy than acute
leukemia.
 II. Etiology of CML :
Not clear yet.
- Cytotoxic drugs ?
- Viruses ?
- Radiation ?
- Pollutants ?
 III. Clinical picture of CML :
Adult & elderly
Male > female
Splenomegaly ( Schuffner VII / VIII )
Sometimes hepatomegaly
Complaints : abd. fullness/dyspneu
abdominal mass
early satiety
 Clinical progression in CML :
I. ChronicPhase
relatively stabil, lasting months / years
II. Accelerated Phase
decreased clin condition, weeks /months
blood smear : increased of blast cells
III. Blast Crisis Phase
worsened condition & laboratory findings
as an acute leukemia
 Pathogenesis of CML :
Majority : Philadelphia chromosome (+)
t (9:22) : resiprocal transversion of genetic

material from chrom 9 to 22 (vv)

new abnormal protein
initiating abnormal proliferation of myeloid cells
in bone-marrow
CML.
Sebuah Kariotip dengan translokasi kromosom 9 dan 22 : Philadelphia chromosome
 IV. Diagnosis of CML :
Clinical pictures : adult/elderly, splenomegaly
Routine blood : Hb low/normal/increased
Platelet normal / increased
WBC quite increased
Blood smear : immature WBC ( blast cells ),
intermediate (rods),mature WBC
granulocytes all are increased
 Peripheral blood smear :
Blast cells < 5 % or
Blast + promyelocytes < 10 %

Other marker : Leukocyte Alkaline Phospatase <

 Bone marrow aspiration :
Increased of BM cellularity ( hypercellularity )
Common BM appearance :
- erythropoiesis normal / increased
- megakaryopoiesis normal / increased
- granulopoiesis : hyperactive
( increased of granulocytes in all of stage
of maturation )
BMA in the CML case ( hypercellularity and increased of granulocytes in all of stage
of maturation)
BMA in the CML case ( hypercellularity and increased of granulocytes in all of stage
of maturation)
 The differences with Acute Leukemia :

Acute leukemia : - monotonous picture

(dominated by blast cells/immature)
- hiatus Leukemicus
( very little amount of intermediate form cells )
 V. Treatment of CML (1) :
1. Glivec : ( Imatinib Mesylate ) per-oral.
Can eradicate Philadelphia chrom.
Very expensive
2. Myleran : ( Busulfan ) per-oral
Potent & cheap, but toxic
3. Hydrea : ( Hydroxy Urea ) per-oral
Fair / not expensive
 Treatment of CML (2) :
4. Alpha Interferon inj :
- imunomodulator & antiproliferative effect
- can be given alone or simultaneously with
Cytosine Arabinoside ( Cytarabin ) inj.
Duration of treatment : 1 4 yr
Relatively very expensive
 Treatment of CML (3) :
New technic :
Peripheral Blood Stem-cell Transplantation ( PBSCT )
Advantages :
- no need operating theatre
- relatively simple & easy procedure
- low cost
- most ideally procedure (?)
 VI. Prognosis of CML :
Depends on : - 1. age (>60 yo)
- 2. splenomegaly (>10 cm bac)
- 3. blast > 3 % in blood smear
or > 5 % in BM
- 4. platelet > 700.000 / mm3
All above are Negative Prognosis Factors
 Life expectancy of CML :
Survival Rate : Median survival 4 yrs
10 % died in the 2nd yr
Yearly after : increased by 20 %
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