Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel

Author:

Tefferi Ayalew1,Thiele Juergen2,Orazi Attilio3,Kvasnicka Hans Michael2,Barbui Tiziano4,Hanson Curtis A.1,Barosi Giovanni5,Verstovsek Srdan6,Birgegard Gunnar7,Mesa Ruben1,Reilly John T.8,Gisslinger Heinz9,Vannucchi Alessandro M.10,Cervantes Francisco11,Finazzi Guido4,Hoffman Ronald12,Gilliland D. Gary13,Bloomfield Clara D.14,Vardiman James W.15

Affiliation:

1. Mayo Clinic, Rochester, MN;

2. Institute of Pathology, University of Cologne, Cologne, Germany;

3. Indiana University School of Medicine, Indianapolis;

4. Ospedali Riuniti di Bergamo, Bergamo, Italy;

5. Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico, San Matteo, Pavia, Italy;

6. M. D. Anderson Cancer Center, Houston, TX;

7. University Hospital, Uppsala, Sweden;

8. Royal Hallamshire Hospital, Sheffield, United Kingdom;

9. Medical University of Vienna, Vienna, Austria;

10. University of Florence, Florence, Italy;

11. Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain;

12. University of Illinois, Chicago;

13. Dana-Farber Cancer Institute, Boston, MA;

14. Ohio State University, Columbus;

15. University of Chicago, IL

Abstract

AbstractThe Janus kinase 2 mutation, JAK2617V>F, is myeloid neoplasm-specific; its presence excludes secondary polycythemia, thrombocytosis, or bone marrow fibrosis from other causes. Furthermore, JAK2617V>F or a JAK2 exon 12 mutation is present in virtually all patients with polycythemia vera (PV), whereas JAK2617V>F also occurs in approximately half of patients with essential thrombocythemia (ET) or primary myelofibrosis (PMF). Therefore, JAK2 mutation screening holds the promise of a decisive diagnostic test in PV while being complementary to histology for the diagnosis of ET and PMF; the combination of molecular testing and histologic review should also facilitate diagnosis of ET associated with borderline thrombocytosis. Accordingly, revision of the current World Health Organization (WHO) diagnostic criteria for PV, ET, and PMF is warranted; JAK2 mutation analysis should be listed as a major criterion for PV diagnosis, and the platelet count threshold for ET diagnosis can be lowered from 600 to 450 × 109/L. The current document was prepared by an international expert panel of pathologists and clinical investigators in myeloproliferative disorders; it was subsequently presented to members of the Clinical Advisory Committee for the revision of the WHO Classification of Myeloid Neoplasms, who endorsed the document and recommended its adoption by the WHO.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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