Publication
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 mutation
Author
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.
Language
English
Source (journal)
Turkish Journal of Haematology
Publication
2014
ISSN
1300-7777
Volume/pages
31:3(2014), p. 239-254
ISI
000341678000005
Full text (Publisher's DOI)
Full text (open access)
UAntwerpen
Faculty/Department
Research group
Publication type
Subject
Affiliation
Publications with a UAntwerp address
External links
Web of Science
Record
Identification
Creation 06.11.2014
Last edited 20.07.2017
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