Polycythemia Vera
Definition
- Dysregulated expansion of the red blood cell mass
Diagnostic Criteria
- Prodromal, pre-polycythemic diagnosis is difficult to make
- Borderline to mild erythrocytosis
- Bone marrow biopsy showing panmyelosis with decreased erythropoietin may be helpful for diagnosis
- Proliferative phase diagnosis requires 2 major and 1 minor or 1 major and 2 minor criteria:
- Major criteria
- Hemoglobin >18.5 g/dl (men) or >16.5 g/dl (women)
- If iron deficient, can be anemic but will still have prominent polychromasia
- JAK2V617F or other functional similar mutation
- Minor criteria
- Hypercellular bone marrow with panmyelosis
- Erythroid hyperplasia often predominates
- Low serum erythropoietin (EPO)
- Endogenous erythroid colony formation in vitro
- Variable excess platelet or leukocyte production
- Post-polycythemic myelofibrosis phase requires both:
- Previous diagnosis of PV
- Bone marrow fibrosis
- Grade 2-3 (coarse diffuse reticulin fibrosis with or without collagen fibrosis, European consensus system, Thiele 2005)
- AND any two of following
- Anemia or sustained loss of phlebotomy/cytoreduction treatment for erythrocytosis
- Leukoerythroblastic blood smear
- Increasing splenomegaly
- Development of >1 of following constitutional symptoms
- >10% weight loss in 6 months
- Night sweats
- Unexplained fever
Dita Gratzinger MD PhD
Tracy I George MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting: 10/23/11
