Philadelphia Chromossome-Negative Myeloproliferative Neoplasms

Includes
  • Essential thrombocythemia
  • Polycythemia vera (PV)
  • Primary myelofibrosis
 
Etiology
  • 90% of patients have somatic phenotypic driver mutation in JAK2 (V617F), CALR or MPL
 
Clinical presentation
  • Thromboembolism common when platelets 400-1000x10^9
  • Cutaneous lesions: purpura, hematomas, livedo reticularis e racemosa, raynaud, urticaria, vasculite de pequenos vasos, úlceras de perna, gangrena, recurrent superficial trhombophlebitis
    • Erythromelalgia: intense burning and paroxysmal bright erythema of the distal extremities
      • Responds well to aspirin
 
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