Table 3.

Effects of therapy on JAK2V617F cell quantity, quality, and clonal expansion

PhlebotomyHydroxyureaRuxolitinibRopeginterferon
Quantity ++ +++ +++ 
Comments Rapid reduction of red cells and blood volume Control of WBC, RBC, and platelets Control of WBC, RBC, and platelets. Most reliable hematocrit control Control of WBC, RBC, and platelets, sustained with molecular responses 
Quality – – 
Comments Reinforces iron deficiency, activates HIF42,46  Selection stress on bone marrow HSC65  Reduced intracellular signaling47  Targets and extinguishes JAK2V617F-positive HSC66  
Clonal suppression – – +++ 
Comments VAF increases ∼1% per year61  Transient reduction in first year, then rebounds VAF increases ∼1% per year60,67  Mild to moderate, but highly variable VAF reduction3,68,69  Consistent reduction in VAF VAF decreases ∼1% per month over 36 months60,61,70,71  
PhlebotomyHydroxyureaRuxolitinibRopeginterferon
Quantity ++ +++ +++ 
Comments Rapid reduction of red cells and blood volume Control of WBC, RBC, and platelets Control of WBC, RBC, and platelets. Most reliable hematocrit control Control of WBC, RBC, and platelets, sustained with molecular responses 
Quality – – 
Comments Reinforces iron deficiency, activates HIF42,46  Selection stress on bone marrow HSC65  Reduced intracellular signaling47  Targets and extinguishes JAK2V617F-positive HSC66  
Clonal suppression – – +++ 
Comments VAF increases ∼1% per year61  Transient reduction in first year, then rebounds VAF increases ∼1% per year60,67  Mild to moderate, but highly variable VAF reduction3,68,69  Consistent reduction in VAF VAF decreases ∼1% per month over 36 months60,61,70,71  

EMH, extramedullary hematopoiesis; RBC, red blood cell; WBC, white blood cell.

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