What are the Polycythemia Vera Study Group (PVSG) diagnostic criteria for polycythemia vera (PV)?

Updated: Aug 20, 2019
  • Author: Srikanth Nagalla, MBBS, MS, FACP; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
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Answer

The Polycythemia Vera Study Group (PVSG) was the first to set rigorous criteria for the diagnosis of polycythemia vera (PV) in the 1970s. With the establishment of polymerase chain reaction (PCR)–based methods for detecting the JAK2 V617F mutation, this may become the first molecular diagnostic marker for PV, similar to BCR/ABL for chronic myelogenous leukemia (CML). However, because of a paucity of centers doing red blood cell mass measurements, demonstrating an elevated red blood cell mass continues to become more difficult.

The diagnostic criteria set by the PVSG are organized into two categories, A and B. The diagnosis of PV is established if all three category A criteria are present, or if criteria A1 plus A2 plus any two criteria from category B are present.

Category A criteria are as follows:

  1. Total red blood cell mass ≥36 mL/kg in males or ≥32 mL/kg in females

  2. Arterial oxygen saturation ≥92%

  3. Splenomegaly

Category B criteria are as follows:

  • Thrombocytosis, with platelet count >400,000/μL

  • Leukocytosis, with a white blood cell count >12,000/μL

  • Increased leukocyte alkaline phosphatase (ALP) >100 U/L

  • Serum vitamin B-12 concentration >900 pg/mL or binding capacity >2200 pg/mL

Total red blood cell mass is measured by labeling the cells with chromium 51 (51Cr). Documentation of an elevated total red blood cell mass with 51Cr-labeled red blood cells and, ideally, an iodine-131 (131I) plasma volume dual technique differentiates true erythrocytosis from pseudoerythrocytosis (decreased plasma volume).

However, the red blood cell mass is becoming difficult to obtain because the 51Cr isotope needed to perform the test is no longer readily available, and institutions willing to perform the test are few as a result of small demand and lack of profit in performing the test.


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