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Diagnosis according to World Health Organization determines the long-term prognosis in patients with myeloproliferative neoplasms treated with anagrelide: Results of a prospective long-term follow-up
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
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2013 (English)In: Hematology, ISSN 1024-5332, E-ISSN 1607-8454, Vol. 18, no 1, 8-13 p.Article in journal (Refereed) Published
Abstract [en]

OBJECTIVES:

During long term follow-up of a cohort of patients with essential thrombocythemia (ET) and polycythemia vera (PV) a higher than expected incidence of myelofibrosis (MF) was noted. In order to test if the explanation could be found in the diagnostic criteria a re-evaluation of diagnosis using the 2008 WHO diagnostic criteria for ET and MF was performed.

METHODS:

This prospective study of 60 patients with ET and PV was set up in 1998 to evaluate the long-term efficacy and tolerability of anagrelide treatment. Bone marrow trephine biopsies were requested from study start, after 2 and 7 years of follow-up. A blinded re-evaluation of the bone marrow trephines was performed. The 2008 WHO bone marrow criteria were used for diagnosis and fibrosis grading.

RESULTS:

Of 40 patients with an initial diagnosis of ET, 21 were confirmed as 'true ET' whereas 17 were reclassified as primary myelofibrosis (PMF) (12 PMF-0, 3 PMF-1, 2 PMF-2) and 2 as myeloproliferative neoplasms of uncertain origin. After 7 years of follow-up, 19 of 21 patients with 'true ET' were alive, none had transformed to MF, leukemia, or myelodysplastic syndrome. In contrast, 4/17 patients reclassified as PMF had died, two patients transformed to myelodysplastic syndrome and 7 patients progressed to overt MF.

DISCUSSION:

We conclude that a blinded re-evaluation of bone marrow trephines from study start and after 7 years of follow-up using 2008 World Health Organization criteria was able to differentiate between true ET and PMF with a marked difference in follow-up outcome.

Place, publisher, year, edition, pages
2013. Vol. 18, no 1, 8-13 p.
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:uu:diva-182582DOI: 10.1179/1607845412Y.0000000023ISI: 000314852700002PubMedID: 22990042OAI: oai:DiVA.org:uu-182582DiVA: diva2:560081
Available from: 2012-10-11 Created: 2012-10-11 Last updated: 2017-12-07Bibliographically approved

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Ejerblad, ElisabethBirgegård, Gunnar

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