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Essential thrombocythemia with high hemoglobin levels according to the revised WHO classification

Leukemia volume 28, pages 2092–2094 (2014)Cite this article

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Differentiation between essential thrombocythemia (ET) and polycythemia vera (PV) persists to be a matter of controversy, particularly in patients who present with increased hemoglobin (HB) levels accompanied by very high platelet counts. Although considered as a ‘diagnostic gold standard’,1 the impact and practicability of red cell mass (RCM) measurements in patients with the clinical differential diagnosis of PV versus ET has to be discussed.2, 3 In this context the diagnostic accuracy of different HB or hematocrit (HCT) threshold values and bone marrow (BM) morphology as surrogate markers for RCM have been considered.4, 5 To capture PV patients with a borderline increase in HB (⩽18.5 g/dl in men and ⩽16.5 g/dl in women), in addition to JAK2V617F or JAK2 exon 12 mutations and characteristic BM biopsy features,6 a lowering of the HB and HCT values was postulated for the prospective revision of the 2008 WHO classification.7 On the basis of the previous studies regarding so-called masked PV, threshold values included a HB level of 16.5 g/dl in men and 16 g/dl for women or a HCT level of 49% in men and 48% in women to be the optimal cutoff levels for distinguishing JAK2V617 ET from masked PV.5 On the other hand, no knowledge exists on how many patients with World Health Organization (WHO)-confirmed ET including consistent BM features present with HB levels exceeding these postulated thresholds, that is, clinically suggesting (early/masked) PV.3, 5

Our experience regarding this critical diagnostic pitfall was based on 891 patients with ET derived from an international study8 (cohort 1) and diagnosed according to the classification proposed by the WHO.9 Moreover, a validation sample (cohort 2) was considered including 248 patients selected from the Frankfurt database again with strictly WHO-defined ET diagnosis.

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Acknowledgements

TB and AMV were supported by a grant from Associazione Italiana per la Ricerca sul Cancro (AIRC, Milano) ‘Special Program Molecular Clinical Oncology 5 × 1000’ to AGIMM (AIRC-Gruppo Italiano Malattie Mieloproliferative).

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Author notes

  1. T Barbui, J Thiele, H M Kvasnicka, A Carobbio, A M Vannucchi and A Tefferi: All authors contributed equally to this work.

Authors and Affiliations

  1. Research Foundation, Papa Giovanni XXIII Hospital, Bergamo, Italy

    T Barbui & A Carobbio

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

    J Thiele

  3. Senckenberg Institute of Pathology, University of Frankfurt, Frankfurt, Germany

    H M Kvasnicka

  4. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

    A M Vannucchi

  5. Hematology Division, Mayo Clinic, Rochester, MN, USA

    A Tefferi

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  1. T Barbui
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Correspondence to T Barbui.

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Barbui, T., Thiele, J., Kvasnicka, H. et al. Essential thrombocythemia with high hemoglobin levels according to the revised WHO classification. Leukemia 28, 2092–2094 (2014). https://doi.org/10.1038/leu.2014.175

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