Download Chronic Myeloid leukemia

Myeloprolifrative disorders
-Chronic Myelogenouse Leukemia
- Primary Poly Cythemia ( vira )
- Essential Thrombocythemia
- Myelofibrose Myeloid Methaplasia
Chronic Myeloid leukemia
Malignant disease of an early
hemopoietic progenitor cell. The clonal
marker is found in all three lineage :
- erythroid
- myeloid
- megakaryocyte
Chronic Myelogeneuse Leukemia
- >90% 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.
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.
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.
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.
Clinical Symptom and Signs
1- Many patients asymptomatic..
2- Splenomegaly & Hypochondrial pain.
3- Fatigue, Weight loss, Sweating
4- Hepatomegaly & abdominal fullness.
5- Gout, bruising , splenic infarct
& priapism.
6- Leukostasis
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.
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.
Classification of Polycythemia
-Essential Polycythemia
-Hypoxic Polycythemia
-Polycythemia due to high Epo.
- Apparent polycythemia