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Chronic Myeloid leukemia

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-The presence of the Ph chromosome in acute leukemia ... Slide 1 Chronic Myeloid leukemia Slide 3 Slide 4 Slide 5 Slide 6 ... – PowerPoint PPT presentation

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Title: Chronic Myeloid leukemia


1
Myeloprolifrative disorders
  • Chronic Myelogenouse Leukemia
  • Primary Poly Cythemia ( vira )
  • Essential Thrombocythemia
  • Myelofibrose Myeloid Methaplasia

2
Chronic Myeloid leukemia
  • Malignant disease of an early
    hemopoietic progenitor cell. The clonal marker
    is found in all three lineage
  • - erythroid
  • - myeloid
  • - megakaryocyte

3
?Chronic Myelogeneuse Leukemia
- gt90 Ph
- 3 phase of disease , Chronic , Accelatory ,
Acute.
- May present with any phase.
- Duration of chronic phase varies ( 3m -23y.
Medium4.5 y
- All cases eventually transform to blast crisis.
4
?Clinical symptoms and signs in CML
-Many patiente asymptomatic
- Fatigue , Weight loss , Sweating ,..
- Splenomegaly may cause abdominal fullness.
- Gout , bruising , spleen infarct , priapism.
- Signs include Splenomegaly Hepatomegaly.
- Occasional Leucostatic signs at presentation.
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Lab findings in CML       1-  20 or 30
percent peripheral blood or bone marrow
blasts     2- Large foci or clusters of
blasts on the bone marrow biopsy .   
3-  Presence of extramedullary blastic
infiltrates (eg, chloroma). These definitions
become important for prognosis, particularly in
patients considered for hematopoietic cell
transplantation, in whom the outcome is better
when transplantation is performed in the chronic
phase.
10
Diagnosis and investigations of CML
FBC blood film (WBC high mainly Neutrophil
Myelocyte
Platelets may be raised clumped on film.
ESR N-APh score low in absence infection.
LDH Urate level are high
BM gross Hypercellularity and Ph on chromosomal
analysis.
Blast count raises with blastic crisis
transformation.
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Other Philadelphia chromosome ve malignancies
The majority of these patients exhibit a
clinical course consistent with CML, including
eventual progression to blast crisis, and
probably represent CML with an atypical initial
presentation . sensitive reverse
transcriptase-polymerase chain reaction (RT-PCR)
assays for detection of the BCR-ABL fusion mRNA
have shown that this product can be detected at
low levels in approximately 50 of Ph-negative
patients with E T the significance of BCR-ABL
transcripts in this setting requires further
investigation. -The Ph chromosome is found in
20 to 30 percent of adults
with acute
B-lymphoblastic leukemia . - 5 to 10 of
childhood B-ALL , and 1 to 2 of adult AML.
-The presence of
the Ph chromosome in acute leukemia
patients strongly correlates with a
poor prognosis.
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Treatment of CML
- Incurable without stem cell Transplantation.
-Hydroxturea in maintenance drug of choice
(controling WBC) 1-1.5g po/d.
rash , mouth ulcer and diarrhoea.
  • INF-a SC may produce cytogenetic response

  • (reducing ve Ph cells).

All patients on IFn-a have longer survival
than Hydroxyurea.
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Classification of Polycythemia
  • Essential Polycythemia
  • Hypoxic Polycythemia
  • Polycythemia due to high Epo.
  • - Apparent polycythemia

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