- Clonal proliferation of pleuripotent stem cells that release cytokines, causing BM fibrosis
 
        
            - Sometimes polycythemia vera, essential thrombocytosis first
 
        
            - Can evolve into AML
 
        
      
     
   
           
        
        
        
          
          
            
  
      
    
    
      
        
            - Average age 67 (17% present at age < 50)
 
        
            - History polycythemia vera or essential thrombocytosis
 
        
      
     
   
           
        
        
        
          
          
            
  
      
        
        
          
            
                - Night sweats
 
            
                - Weight loss
 
            
                - Fever
 
            
          
         
        
        
       
    
    
      
        
            - Generalized fatigue
 
        
            - Sob, doe→ from anemia
 
        
            - Abdominal discomfort, early satiety from hepato-splenomegaly→ sites of extra medullary hematopoeisis
 
        
            - Thrombotic events: portal vein thrombosis, DVT, PE
 
        
      
     
   
           
        
        
        
          
          
        
       
      
      
  
  
    
      - Often elevated WBCs, sometimes w/blasts; as develop BM failure--> anemia
 
    
      - Often + JAK2 mutation
 
    
      - Bone marrow bx to make dx