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Classification of myeloproliferative disorders in the JAK2 era: is there a role for red cell mass?

Leukemia volume 22, pages 452–453 (2008)Cite this article

The discovery of JAK2 mutation1 (JAK2V617F) has led to the proposal of new algorithms to diagnose and classify Ph-negative myeloproliferative disorders (MPDs).2 Separation of polycythemia vera (PV) from essential thrombocythemia (ET) could become less clear, especially in JAK2V617F patients, if one considers that mutated PV and ET are similar conditions.3, 4 Yet, no prospective data support this assumption. In particular, both the short-term risk of thrombosis and long-term risk of evolution to leukemia have not been shown to be similar in JAK2-mutated ET and PV.3 The main biological difference between PV and ET lies in absolute erythrocytosis, formally demonstrated by red cell mass (RCM) measurement. The current WHO criteria for PV diagnosis require either increased RCM above 125% of predicted value or hemoglobin (Hb) level above 18.5 g/dl in men and 16.5 g/dl in women. In addition, some investigators have suggested setting a threshold for hematocrit (Ht) at 52% in men and 48% in women to diagnose PV in JAK2-mutated patients.5 In contrast, diagnosis of ET requires excluding PV. Using Hb or Ht values for this purpose could avoid RCM measurement if those parameters were reliable for detecting erythrocytosis in MPD.2, 6

This study aimed to evaluate the prevalence and prognostic impact of erythrocytosis in MPD patients classified as ET on peripheral blood counts, by reviewing all patients referred to two nuclear medicine departments for RCM determination between 1996 and 2006. Of 566 patients tested (after eliminating causes of reactive thrombocytosis), 71 had thrombocytosis and Hb and Ht below the values used for PV diagnosis (as defined above), and were therefore diagnosed with ET before RCM measurement.

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Authors and Affiliations

  1. AP-HP, Hôpital Saint-Louis, Unité de Biologie Cellulaire, Paris, France

    B Cassinat, C Chomienne & J-J Kiladjian

  2. AP-HP, Hôpital Avicenne, Service de Médecine Nucléaire, Bobigny, France

    C Laguillier & V de Beco

  3. AP-HP, Hôpital Avicenne, Service d'Hématologie Clinique and Université Paris 13, Bobigny, France

    C Gardin, S Burcheri & P Fenaux

  4. PV-Nord, Hôpital Saint-Louis, Paris, France

    B Cassinat, P Fenaux, C Chomienne & J-J Kiladjian

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  1. B Cassinat
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  2. C Laguillier
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  3. C Gardin
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  8. J-J Kiladjian
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Correspondence to J-J Kiladjian.

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Cassinat, B., Laguillier, C., Gardin, C. et al. Classification of myeloproliferative disorders in the JAK2 era: is there a role for red cell mass?. Leukemia 22, 452–453 (2008). https://doi.org/10.1038/sj.leu.2404908

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