Title
Clinical, pathological and molecular features of the chronic myeloproliferative disorders : MPD 2005 and beyond Clinical, pathological and molecular features of the chronic myeloproliferative disorders : MPD 2005 and beyond
Author
Faculty/Department
University Hospital Antwerp
Publication type
article
Publication
Subject
Human medicine
Source (journal)
Hematology (Amsterdam, Netherlands)
Source (book)
30th World Congress of the International-Society-of-Hematology, 2005, Istanbul, TURKEY
Volume/pages
10(2005) :s:[1] , p. 215-223
ISSN
1024-5332
ISI
000232835500059
Carrier
E
Target language
English (eng)
Full text (Publishers DOI)
Affiliation
University of Antwerp
Abstract
The combined use of bone marrow histopathology, biomarkers and clinical features has the potential to diagnose, stage and distinguish early and overt stages of ET, PV and idiopathic myelofibrosis, that has an important impact on prognosis and treatment of MPD patients. As the extension of the PVSG and WHO for ET, PV and agnogenic myeloid metaplasia (AMM), a new set of European clinical and pathological (ECP) criteria clearly distinct true ET from early or latent PV mimicking true ET, overt and advanced polycythemia vera (PV), and from thrombocythemia associated with prefibotic, early fibrotic stages of chronic megakaryocytic granulocytic metaplasia (CMGM) or chronic idiopathic myelofibrosis (CIMF). Cases of atypical MPD and masked PV are usually overlooked by clinicians and pathologists. Bone marrow biopsy will not differentiate between post-PV myelofibrosis versus so-called classical agnogenic myeloid metaplasia. The recent discovery of the JAK2 V617F mutation can readily explain the trilinear megakaryocytic, erythroid and granulocytic proliferation in the bone marrow, but also the etiology of the platelet-mediated microvascular thrombotic complications at increased platelet counts and red cell mass in essential thrombocythemia and polycythemia vera.
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