Myeloprolifrative disorders -Chronic Myelogenouse Leukemia - Primary Poly Cythemia ( vira ) -...
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Myeloprolifrative disorders Myeloprolifrative disorders -Chronic Myelogenouse Leukemia - Primary Poly Cythemia ( vira ) - Essential Thrombocythemia - Myelofibrose Myeloid Methaplasia
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Transcript of Myeloprolifrative disorders -Chronic Myelogenouse Leukemia - Primary Poly Cythemia ( vira ) -...
Myeloprolifrative disordersMyeloprolifrative 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 LeukemiaChronic 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 CMLClinical 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 CMLDiagnosis 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 SignsClinical 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 CMLTreatment 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.
Treatment
Incurable without BMTHydroxyurea 1-1.5gPO/d.
Glivic cap 3cap/d
IFN-a
Treatment
Incurable without BMTHydroxyurea 1-1.5gPO/d.
Glivic cap 3cap/d
IFN-a
Classification of PolycythemiaClassification of Polycythemia
-Essential Polycythemia
-Hypoxic Polycythemia
-Polycythemia due to high Epo.
- Apparent polycythemia
