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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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