Essential thrombocythaemia
Description
- MPN with sustained clonal thrombocytosis and megakaryocytic proliferation
- The most indolent MPN - near-normal life expectancy in low-risk young patients
- Presentation
- Incidental raised platelet count (~50-60%)
- Microvascular/vasomotor: erythromelalgia, acral paraesthesia, migraine-like headache, transient visual disturbance, atypical chest pain
- Thrombosis - arterial > venous; sometimes at unusual sites (splanchnic, cerebral venous sinus)
- Bleeding at extreme counts - acquired von Willebrand syndrome
- Mild splenomegaly ~20-50%
- *Two mimics must be excluded before the diagnosis stands*
- Prefibrotic primary myelofibrosis - looks identical clinically, worse outcome, distinguished only on trephine
- Masked polycythaemia vera - concurrent iron deficiency suppressing the Hb
6 more sections, plus exam facts
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