Table 2

Core set of conceptual criteria to be used for defining response in ET and PV

Response category/essential thrombocythemiaResponse category/polycythemia vera
Clinicohematologic  
    Reduction/normalization of platelet count Reduction of phlebotomy requirement to maintain the hematocrit at the target level 
    Resolution of disease-related symptoms* Reduction/normalization of platelet count 
    Reduction/normalization of splenomegaly on imaging Reduction/normalization of white blood cell count 
    Reduction/normalization of white blood cell count Reduction/normalization of splenomegaly on imaging 
 Resolution of disease-related symptoms* 
Molecular  
    Reduction of any specific molecular abnormalities Reduction of any specific molecular abnormalities 
Histologic  
    Bone marrow histologic remission defined as the disappearance of megakaryocyte hyperplasia Bone marrow histologic remission defined as the presence of age-adjusted normocellularity and no reticulin fibrosis 
Response category/essential thrombocythemiaResponse category/polycythemia vera
Clinicohematologic  
    Reduction/normalization of platelet count Reduction of phlebotomy requirement to maintain the hematocrit at the target level 
    Resolution of disease-related symptoms* Reduction/normalization of platelet count 
    Reduction/normalization of splenomegaly on imaging Reduction/normalization of white blood cell count 
    Reduction/normalization of white blood cell count Reduction/normalization of splenomegaly on imaging 
 Resolution of disease-related symptoms* 
Molecular  
    Reduction of any specific molecular abnormalities Reduction of any specific molecular abnormalities 
Histologic  
    Bone marrow histologic remission defined as the disappearance of megakaryocyte hyperplasia Bone marrow histologic remission defined as the presence of age-adjusted normocellularity and no reticulin fibrosis 
*

Disease-related symptoms: microvascular disturbances, pruritus, headache.

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