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DR Medhat MRCP

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• Diagnosis:<br />

Philadelphia is confirmatory.<br />

Peripheral blood film :<br />

- Leukocytosis (granulocytes) in all stages of differentiation within the myeloid<br />

linage.<br />

- Basophilia is important diagnostic marker especially when Philadelphia is<br />

هام جدا absent.<br />

- Monocytopenia<br />

Neutrophil alkaline phosphatase. (differentiate it from leukemoid reaction)<br />

Bone-marrow hypercellularity with<br />

myloid-erythroid ratio.<br />

Granulocytes at different stages of<br />

diferentiation<br />

- DD of BM failure with a large number of circulating myeloid series’ cells :<br />

1) Chronic myelogenous leukaemia (CML) or<br />

2) Myelofibrosis<br />

- The C/P and routine investigations are often the same in both conditions.<br />

- The diagnosis of myelofibrosis needs a trephine biopsy as the aspirate is often ‘dry’.<br />

• Management :<br />

1) Imatinib : Now is the 1 st line treatment (mechanism : inhibitor of tyrosine kinase)<br />

2) Hydroxyurea (also used in PRV, painful attacks in sicklers and as antiretroviral in HIV)<br />

3) Interferon- α.<br />

4) Allogenic bone marrow transplant<br />

Philadelphia translocation, t(9:22)<br />

Good prognosis in CML.<br />

Poor prognosis in AML & ALL.<br />

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