Title
The European clinical, molecular, and pathological (ECMP) criteria and the 2007/2008 revisions of the World Health Organization for the diagnosis, classification, and staging of prefibrotic myeloproliferative neoplasms carrying the <tex>$JAK2^{V617F}$</tex> mutation The European clinical, molecular, and pathological (ECMP) criteria and the 2007/2008 revisions of the World Health Organization for the diagnosis, classification, and staging of prefibrotic myeloproliferative neoplasms carrying the <tex>$JAK2^{V617F}$</tex> mutation
Author
Faculty/Department
Faculty of Medicine and Health Sciences
University Hospital Antwerp
Publication type
article
Publication
Subject
Human medicine
Source (journal)
Turkish Journal of Haematology
Volume/pages
31(2014) :3 , p. 239-254
ISSN
1300-7777
ISI
000341678000005
Carrier
E
Target language
English (eng)
Full text (Publishers DOI)
Affiliation
University of Antwerp
Abstract
Objective: The prefibrotic stages of JAK2(V617F) essential thrombocythemia (ET) and JAK2(V617F) polycythemia vera (PV) can easily be diagnosed clinically without use of bone marrow biopsy histology. We assessed the 2008 WHO and European Clinical, Molecular, and Pathological (ECMP) criteria for the diagnosis of myeloproliferative neoplasms (MPNs). Materials and Methods: Studied patients included 6JAK2(V617F)-mutated ET and 4 PV patients during long-term follow-up in view of critical analysis of the literature. The bone marrow biopsy histology diagnosis without use of clinical data was PV in 7 (of which 3 were cases of ET with features of early prodromal PV) and classical PV in 4. Results: The ECMP criteria distinguish 3 sequential phenotypes (1, 2, or 3) of JAK2(V617F)-mutated ET: normocellular ET-1; ET-2, with clinical and bone marrow features of PV (prodromal PV), and ET-3, with hypercellular dysmorphic megakaryocytic and granulocytic myeloproliferation (ET.MGM). The 3 patients with ET-2 or prodromal PV developed slow-onset PV after a follow-up of about 10 years. Bone marrow biopsy histology differentiates MPNs of various molecular etiologies from all variants of primary or secondary erythrocytoses and thrombocytoses with sensitivity and specificity of near 100%. Conclusion: Normocellular ET (WHO-ET), prodromal PV, and classical PV show overlapping bone marrow biopsy histology features with similar pleomorphic clustered megakaryocytes in the prefibrotic stages of JAK2(V617F) mutated MPN. Erythrocytes are below 6x10(12)/L in normocellular ET and prodromal PV, and are consistently above 6x10(12)/L in classical PV and at the time of transition from prodromal PV into classical PV. Red cell count at a cut-off level of 6x10(12)/L separates ET from PV and obviates the need for red cell mass measurement when bone marrow histology and JAK2(V617F) mutation screening are included in the diagnostic work-up of MPNs.
Full text (open access)
https://repository.uantwerpen.be/docman/irua/b4d7f7/8504.pdf
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