megakaryoblastic leukaemia

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megakaryoblastic leukaemia

A rare clonal proliferation of platelet stem cells arising in severe myelofibrosis, which is defined by the presence of 30+% blasts in the bone marrow. It is more common in patients with Down syndrome.
 
Clinical findings
Pallor, weakness, excess bleeding, anaemia, leukopaenia.
 
Diagnosis
Immunocytologic studies of von Willebrand factors and lineage-specific glycoproteins (GPIb, IIb/IIIa or IIIa).

Prognosis
Dismal in the sporadic cases and in children with the translocation t(1;22)(p13;q13); the remaining third of patients (with Down syndrome) have a relatively good prognosis.