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Pathology
Malignant tumour of the pleuripotent stem cell which produces excessive numbers of mature granulocytes, eosinophils and basophils
Characterised by the presence of the Philadelphia chromosome
Aetiology
Unknown, rarely caused by ionising irradiation incidence increases with age
Signs
Bruising, petechiae, splenomegaly, hepatomegaly
Symptoms
Fever, weight loss, malaise, night sweats
Investigations
Bloods: FBC – Increased WBC and platelets
Bone Marrow Aspirate: Hypercellular and increased myeloid cells
Molecular Genetics: Assess for Philadelphia Chromosome
Bone Marrow Trephine: Rule out fibrosis
Treatment
Medical: Tyrosine kinase inhibitors, Stem cell transplantation
Prognosis
Improving with advent of tyrosine kinase inhibitors, 84% survival at five years post-diagnosis
Modest increased infection risk, progression to accelerated phase, acute leukaemia or myelofibrosis
Key Facts
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Key References
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