Criteria of Tefferi and Barbui for the Diagnosis of Polycythemia Vera

 

Patient selection: persistent unexplained erythrocytosis

 

Parameters:

(1) serum erythropoietin

(2) clinical findings for polycythemia vera

(3) laboratory findings in polycythemia vera

(4) bone marrow findings

 

Clinical findings suggesting polycythemia vera (PV) include:

(1) splenomegaly

(2) thrombosis

(3) aquagenic purpura

(4) erythromelalgia

 

Laboratory findings suggesting polycythemia vera (PV) include:

(1) thrombocytosis

(2) unexplained leukocytosis

(3) increased leukocyte alkaline phosphatase score

 

Bone marrow findings suggestive of polycythemia vera include:

(1) hypercellularity, atypical erythroid and megakaryocytic hyperplasia, and decreased bone marrow iron stores during polycythemic stage; myelofibrosis with myeloid metaplasia in spent phase

(2) Janus kinase 2 (JAK2) V617F mutation present (may be seen in other myeloproliferative disorders, so not specific for polycythemia vera)

 

Serum Erythropoietin	Clinical and Laboratory Findings	Bone Marrow Findings	Diagnosis
low	NA	one or both features	PV
low	NA	neither	uncertain
normal	present	one or both features	PV
normal	present	neither	uncertain
normal	absent	NA	uncertain
increased	NA	NA	PV unlikely

 

Patient with uncertain diagnosis should be monitored by one or more of the following:

(1) repeat CBC in 3 months

(2) mutation screening for JAK2-V617F