Polycythaemia vera
Description
- JAK2-driven MPN with panmyelosis - red cells dominate, but WCC and platelets rise too
- The management target is thrombosis, not the haematocrit for its own sake
- Presentation
- Incidental raised Hb/Hct on an FBE (commonest)
- Aquagenic pruritus after a hot shower - near-pathognomonic
- Erythromelalgia, plethora, conjunctival suffusion, headache, tinnitus, blurred vision
- Thrombosis at an unusual site - Budd-Chiari, portal or splanchnic vein, cerebral venous sinus
- Gout, peptic ulceration (histamine), splenomegaly (~30-40%)
- *Post-PV myelofibrosis and AML are the two ways it ends*
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